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Page 1: today’s research, tomorrow’s medicinekidsresearch.med.usyd.edu.au/about/reports/annual... · Stream 2 - Tissue Engineering and Bone Repair Orthopaedic Research and Biotechnology

today’s research, tomorrow’s medicine

Annual Report 2008-2009

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2 Annual Report 2008-2009

THANK YOU

Many thanks to the children featured in this report: Cameron, Thomas, Bradley, Jacob, Courtney, Demi Lee, Ebony, Bridget, William, Yanni, Rachel and Jack.

Thank you also to the following staff members of the Kids Research Institute for their help: Stephen Alexander, Louise Baur, Nadia Badawi, Robert Booy, Carolyn Broderick, Joshua Burns, Laurence Cantrill, Dan Catchpoole, John Christodoulou, Chris Cowell, Cheryl Jones, Belinda Kramer, David Little, Renjing Liu, Sloane Madden, Angie Morrow, Charmaine Tam, Karen Waters and Nick Wood.

Special thanks to Amelia Hill of the Research Office and Community Relations and Marketing.

CONTENT Helen Signy, The WriteMedia Network

GRAPHIC DESIGN AND LAYOUTAlice Pennington Design

PHOTOGRAPHYPaul De Sensi

PRINTING AND BINDINGGeckoPrint

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3 Annual Report 2008-2009

Annual Report 2008-2009

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4 Annual Report 2008-2009

ContentsForeword 6

Our organisation 8

Our facilities 9

Our committees 10

Research Council 12

Finances 14

Stream 1 - Neurosciences and Mental Health

Institute of Neuroscience and Muscle Research 18

Department of Adolescent Medicine and Eating Disorders 19

Children’s Hospital Education Research Institute (CHERI) 19

Developmental Cognitive Neuropsychology Research Unit (DeCOG) 20

Stream 2 - Tissue Engineering and Bone Repair

Orthopaedic Research and Biotechnology Unit 22

Children’s Hospital Burns Research Institute (CHBRI) 23

Stream 3 - Cancer Biology

Oncology Research Unit 25

Stream 4 - Genetics, Gene Therapy and Genomics

Human Genome Research Program 28

Gene Therapy Research Unit 30

Stream 5 - Obesity, Metabolism and Nutrition

Institute of Endocrinology and Diabetes 30

Obesity Research Group 31

Children’s Hospital Institute of Sports Medicine 33

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5 Annual Report 2008-2009

Stream 6 - Renal Medicine and Transplantation

Centre for Kidney Research 35

Stream 7 - Infectious Disease and Immunology

Centre for Perinatal Infection Research 37

National Centre for Immunisation Research and Surveillance (NCIRS) 38

Department of Allergy and Immunology 38

Department of Infectious Diseases and Microbiology 39

Stream 8 - Clinical Sciences and Health Services Delivery

Australian Paediatric Surveillance Unit (APSU) 41

Kids Heart Research 42

Centre for Trauma Care, Prevention, Education and Research 43

Respiratory Medicine Research Group 43

SIDS and Sleep Apnoea Research Group 44

Neonatology and the Grace Centre for Newborn Care Research Unit 46

Department of Nuclear Medicine 46

Nursing Research and Practice Development Unit 48

The Children’s Hospital at Westmead Clinical School 48

Centre for Evidence-Based Paediatrics, Gastroenterology and Nutrition 49

Rehabilitation Research Group 50

Staff and Students 52

Grants 62

Publications 66

Our Supporters 77

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6 Annual Report 2008-2009

Dr Antonio Penna Chief Executive, The Children’s Hospital at Westmead

Research is integral to everything we do here at The Children’s Hospital at Westmead. Together with clinical

care, advocacy and education, research forms a critical pillar of our mission to treat sick children.

Our researchers are in a unique position because many of them see sick children every day. What they encounter in their dealings with patients inspires the research work that they do and much of their research is then translated back to improve the lives of the children in our wards.

Our Hospital has a long and distinguished history of world-class medical research, from the discovery of the impact of Rubella on the developing foetus by Dr Norman Grigg in 1942 to our exciting work involving genetics and the causes of disease today. The number of research staff has increased from 40 when the research division was established in 1996 to more than 250 people now working in modern facilities opened in 2005.

This investment in researchers with vitality and passion has paid off. On our campus we now have a world-class paediatric research facility. We collaborate with pre-eminent research centres in Australia and overseas and we attract some of the best professionals from around the globe.

Now it is time for our research unit to develop its own identity, including the important step of renaming our research division the Kids Research Institute.

Rebranding our research arm will lead to two things. It will enhance our research capacity by increasing funding opportunities from government and private donors and it will establish our campus as a beacon on the hill for paediatric research in Australia.

The vision of the Kids Research Institute is to facilitate research across New South Wales, to establish a formal network across the state and provide resources to enhance the work of other clinicians. Through joint collaboration, multi-site research and support for satellite research groups, our impact as a centre for paediatric research excellence will extend far beyond our laboratory walls.

As we build on our strong foundations we hope to increase our capacity for clinical translational research over the next two years with investment in new facilities and increased staff.

I am very proud of the research achievements at The Children’s Hospital at Westmead. I would like to take this opportunity to thank every one of our staff members who is involved in research for their outstanding level of expertise, passion and dedication. Their valuable contributions are achieving better outcomes for sick children throughout Australia and internationally.

Foreword

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7 Annual Report 2008-2009

Clinical Associate Professor Chris Cowell, Acting Director, Kids Research Institute

The Kids Research Institute is dedicated to improving health outcomes and driving excellence in health for children. Our

researchers are involved in all levels of research, from basic sciences in the laboratory to clinical and population health research. Our chief aim is to translate our findings into better health for sick children.

Our research output has increased significantly in the past year. A total of 422 papers were published in 2008, up from 353 in 2007. Our grant income this year was more than $9.8 million, an increase of 30% over two years.

Important research achievements have been made in a wide variety of areas. Our researchers have made ground-breaking advances in the treatment of muscular dystrophy and have discovered significant cellular processes that lead to the healing of bones and burns. Other exciting developments include genetically modifying children’s bone marrow stem cells to make them resistant to high levels of chemotherapy with the hope of improving treatment outcomes in children with brain tumours. The gene responsible for the devastating genetic disorder Arts Syndrome has been identified, leading to new therapy.

Other vital work involves looking at how the fat cells of children change as they grow and making advances in the prevention of obesity and diabetes complications. We are learning more about children’s hearts and the effects of surgery on newborn babies.

In the area of population health research, our scientists played a crucial role in the tracking of the

H1N1 swine influenza pandemic and developing a vaccine for children. Our research findings into children’s accidents is informing Government policy.

In the past year, our capacity for clinical research and clinical trials has been significantly enhanced with the formation of The Australian Children’s Clinical Trials Centre. Together with the University of Sydney, we will be a leader in the provision of infrastructure, support and education to enable clinicians to conduct clinical trials in children and develop innovative treatments and achieve better outcomes.

Our facilities continue to provide an invaluable resource at The Children’s Hospital at Westmead. The Kerry Packer Building, opened on the Westmead campus in 2005, comprises basic laboratories including an imaging suite; a transgenic facility; a Human Movement Laboratory that facilitates research for children with ambulatory disorders, and the Gene and Cell Medicine Facility which is used to modify human cells for gene therapy. We are pleased that this facility was awarded substantial infrastructure funding from the Commonwealth and State governments as part of the Westmead Research Hub cell-based therapies consortium.

Research underpins everything we do as clinicians. We will continue to enhance our understanding of the basis of childhood disease; develop cures and preventions for childhood disease; build up our clinical trials capacity, and incorporate evidence-based health care into clinical practice.

As the Kids Research Institute grows and develops, we are excited about achieving our ultimate aim of turning today’s research into tomorrow’s medicine for children.

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8 Annual Report 2008-2009

Our organisationOur VisionTo be a global leading translational research centre for children.

Our MissionTo improve health outcomes and drive excellence in children’s health by:•Enhancing understanding of the causes of childhood disease

•Developing cures and preventions for childhood disease

•Building capacity to conduct clinical trials in children

•Incorporating evidence-based health care into clinical practice and health outcomes

About the Kids Research InstituteThe Kids Research Institute is the research arm of The Children’s Hospital at Westmead. It is housed in the Kerry Packer Building on the Hospital campus, near Parramatta in Sydney.

Its activities comprise basic science, clinical science and population health research, with research imperatives largely driven by the researchers’ exposure to clinical and population health issues in the course of their daily work with children in the Hospital.

Research areas are broad ranging and researchers are encouraged to collaborate and share their knowledge to provide a multidisciplinary approach to improving child health.

The Institute now comprises more than 250 research and support staff working across eight streams: neurosciences and mental health; tissue engineering and bone repair; cancer biology; genetics, gene therapy and genomics; obesity and metabolism; renal medicine and transplantation; infectious disease and immunology, and clinical sciences and health services delivery.

There is a major emphasis on investigating the efficacy of drugs and treatments suitable for children through conducting clinical trials. This enables new findings made in the laboratory to be tested and translated into new therapies and practices that can be applied as quickly as possible.

The Kids Research Institute is a major contributor to the Westmead Research Hub, a co-operative venture which enables resources and infrastructure to be shared by biomedical and clinical research facilities across the Westmead campus.

Sharing research ideas and projects has been a key success factor for the Kids Research Institute and many of the researchers collaborate with other prestigious national and international medical and educational institutions, including the University of Sydney, Children’s Medical Research Institute, Westmead Millennium Institute and many other institutions in Australia and overseas.

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9 Annual Report 2008-2009

Our facilitiesThe Australian Children’s Clinical Trials Centre (ACCTC)The ACCTC serves all clinical investigators and industry partners who wish to conduct paediatric clinical trials at The Children’s Hospital at Westmead. International, national and local clinical trials are currently underway in a range of areas, including childhood cancer, neuromuscular disease, eating disorders, obesity and diabetes, genetic metabolic disorders, vaccinations for infectious disease, cystic fibrosis, sleep disorders, kidney disease, tissue engineering and health services delivery. The ACCTC‘s services include protocol development, assistance with funding and regulatory approval applications, biostatistical support, in-house training and templates for managing clinical trials to good clinical practice standards.

Human Movement LaboratoryThe Human Movement Laboratory is a large research space where researchers can assess the function and physical capacity of children who are participating in research projects and clinical trials. Specifically, the laboratory is used for capturing and recording measures pertaining to muscle strength (dynamometry), barefoot pressure analysis (pedobarography), walking ability (instrumented gait analysis), breathing and cardiac function, and tasks relating to co-ordination, balance, endurance and power. Data collected from children in the Human Movement Laboratory is processed and analysed to understand how a disease or disability can affect a child’s function and how an intervention may help a patient.

Tumour Bank The Tumour Bank of The Children’s Hospital at Westmead was established with the specific intention of encouraging and facilitating research into cancer in children. It supports research into childhood malignancies by collecting and storing tumour samples, where possible with matched control blood or tissue samples; blood samples from children with malignancies, and serial collections of blood, bone marrow and cerebrospinal fluid from children with leukaemia. These samples are made available to researchers both at The Children’s Hospital at Westmead, nationally and internationally to facilitate research to reduce the incidence of cancer and to improve the outlook of affected children.

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10 Our committees

Our committees

Research Council

ChairJohn Dunlop AM

SecretaryAnne O’Neill (to January 2009)Amelia Hill (from February 2009)

MembersDr Antonio PennaDaniel PetreProfessor David HandelsmanWendy Haigh

Ex-officioAssociate Professor Chris CowellProfessor Ian AlexanderProfessor Kathryn NorthProfessor Peter McIntyre

Research Executive

ChairAssociate Professor Chris Cowell

SecretaryAnne O’Neill (to January 2009)Amelia Hill (from February 2009)

Human Research Ethics Committee

ChairProfessor Kevin Gaskin (to February 2009)Margaret Kelly (from February 2009)

SecretaryCarolyn Casey (to September 2008)Eleanor Thackray (from September 2008)

Minutes SecretaryJessica Moller

Community Members

LaypeopleRuth BurleighMatthew Campbell

Board RepresentativeDr Stuart Dorney

LawyerIan ButcherThe Hon. James Wood

Minister of ReligionSr Patricia Bolster

Professorial Care MemberDr Peter Cooper

Research Members

External ResearcherHelen Slatyer (to December 2008)Catherine McMahon (from May 2009)

Basic/Laboratory ResearchDr Bruce BennettsDr Nicole Graf

Anaesthetic/Surgical ResearchDr John Harvey

Nursing Research/Allied HealthMargaret Kelly (to February 2009)

Oncology AdvisorDr Luce Dalla-Pozza (attendance for COG protocols only)

Clinical Trials PharmacistPathma Joseph

Intellectual Property Committee

ChairDr Antonio Penna

SecretaryAnne O’Neill (to January 2009)Amelia Hill (from February 2009)

MembersWendy HaighAssociate Professor Chris CowellAssociate Professor David LittleProfessor Ian AlexanderDr James McCauley Dr Ralph Hanson (to December 2008)Professor Valerie Wilson

In AttendancePaul Field, Bio-Link Australia Pty. Ltd

Scientific Advisory Committee

ChairmanAssociate Professor Chris Cowell

SecretaryCarolyn Casey (to September 2008)Eleanor Thackray (from September 2008)

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11

Our committees

Minutes SecretaryJessica Moller

MembersProfessor Kevin Gaskin (observes) (to February 2009)Margaret Kelly (observes) (from February 2009)Dr Jonathan EganAssociate Professor Stephen Alexander (to June 2008)Donna Rose (to June 2008)Dr Melanie WongDr Gabrielle Williams Professor Val WilsonDr Patrina CaldwellDr Belinda BartonDr Luce Dalla-PozzaDr Hiran SelvaduraiPathma JosephDr Nick Wood (from December 2008)Professor Robert Booy (from February 2009)Dr Davinder Singh (from February 2009)

Radiation SafetyDr James McCauley (to November 2008)Yvette Wilson (from November 2008)

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12 Our committees

John Dunlop AM (Chair)Mr Dunlop has served as a board member of The Children’s Hospital at Westmead since 1973 including terms as President, Honorary Treasurer and Vice President. He is an external member of a number of The Children’s Hospital at Westmead’s committees as well as a Director of the Children’s Medical Research Institute and Chairman of the Hospitals’ Contribution Fund of

Australia Limited. Formerly Managing Director of Edwards, Dunlop & Company Limited, Mr Dunlop was a director of Health Super Pty Ltd and Health Super Financial Services Pty Ltd between 2000 and 2007. In 1987, Mr Dunlop was appointed a Member of the Order of Australia in recognition of his work for child health.

Anne O’Neill (Secretary, until January 09) Supported by Ms Amelia HillMs O’Neill was the head of Research Administration and Operations at The Children’s Hospital at Westmead and is co-convenor of the ACT/NSW Chapter of the Australasian Research Management Society. At the Hospital, she was responsible for building high performance research support teams,

supporting research of high quality and impact, enabling translational research and facilitating strategic planning for paediatric research. Ms O’Neill moved to NSW Office for Science and Medical Research (OSMR) in January 2009 as Associate Director of Medical Research.

Professor Ian Alexander Professor Alexander is Head of the Gene Therapy Research Unit at the Kids Research Institute, a joint initiative with the Children’s Medical Research Institute. His specific interests include virus-mediated gene transfer with a focus on target organs including the liver and bone marrow. Professor

Alexander’s team became the first in Australia to treat a genetic disease (SCID-X1) by gene therapy and he is recognised as a leader in the establishment of this exciting field in Australia. He was elected the inaugural president of the Australasian Gene Therapy Society in 2001, was appointed Chair of the National Health and Medical Research Council’s Cellular Therapies Advisory Committee in 2007, and joined the Editorial Board of HumTan Gene Therapy in 2009.

Associate Professor Christopher Cowell Associate Professor Cowell is Acting Director of the Kids Research Institute at The Children’s Hospital at Westmead as well as Head of the Institute of Endocrinology and Diabetes. He has been Director of Clinical Research at the Hospital since 2005, a part-time position created to help

advocate and develop clinical research and its infrastructure. A Clinical Associate Professor of the University of Sydney, Associate Professor Cowell trained as a paediatric endocrinologist in Toronto and Sydney and has extensive clinical experience in diabetes, growth, obesity-related metabolic syndrome and disorders of bone metabolism. His major research interests are the prevention of metabolic complications of obesity in teenagers, and the effects of disease states on bone mass and bone geometry.

Ms Wendy Haigh Ms Haigh joined The Children’s Hospital at Westmead in August 2006 as Director of Finance and was appointed Director of Finance and Corporate Services in May 2007. She has a successful track record in finance, having held a number of senior management positions across both the private

and public sectors. Ms Haigh qualified as a chartered accountant with PricewaterhouseCoopers in Britain before transferring to Sydney in 1984. She is a Fellow of the Institute of Chartered Accountants of England and Wales, an Associate of the Institute of Chartered Accountants of Australia, and a member of the Australian Institute of Company Directors.

Professor David Handelsman Professor Handelsman is Australia’s first Professor of Andrology (1996), inaugural Professor/Director of the ANZAC Research Institute (1998), Head of the Andrology Department at Concord Hospital (1999), and Associate Dean (Research Strategy) of the Sydney Medical School.

Research Council

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13 Our committees

Research Council

Over 30 years he has published 350 scientific papers, received an NHMRC Overseas Fellowship and Wellcome Senior Research Fellowship, worked in the USA and Germany, and has served on numerous research and health policy advisory bodies. He was awarded the RACP’s Susman Prize (1994), the inaugural AMA Men’s Health Award (2003) and is an Honorary Life Member, Endocrine Society of Australia (2008). He has served the Editorial Boards of 12 journals, been an invited reviewer for 97 peer-reviewed scientific journals and contributed to NHMRC peer-review for over 20 years, serving on all types of grant committees.

Professor Peter McIntyreProfessor McIntyre is Director of the National Centre for Immunisation Research and Surveillance of Vaccine Preventable Diseases (NCIRS) at the Kids Research Institute, and a Senior Staff Specialist in Infectious Diseases at The Children’s Hospital at Westmead. He has broad interests in vaccines

and vaccine preventable diseases, as well as vaccine preventable diseases in Aboriginal and Torres Strait Islander people. He has been Chief Investigator for a number of vaccine trials, most recently a trial of the first dose of pertussis vaccine at birth rather than two months of age. He is a member of the Australian Technical Advisory Group on Immunisation (ATAGI), the Communicable Diseases Network of Australia (CDNA) and the National Immunisation Committee (NIC). He has been an invited speaker at many international and national meetings and is the author of more than 150 scientific papers and book chapters.

Professor Kathryn NorthProfessor North is the Douglas Burrows Professor of Paediatrics and Associate Dean of The Children’s Hospital at Westmead Clinical School, within the Faculty of Medicine at The University of Sydney. Trained as a paediatric physician, neurologist and clinical geneticist, she completed

a postdoctoral fellowship at Harvard Medical School before returning to Australia in 1995 as the recipient of the Children’s Hospital Research Career Development Award. At The Children’s Hospital at Westmead, Professor North runs the Neurogenetics Clinical Service and is Head of the Institute for Neuroscience and Muscle Research at the Kids Research Institute. Professor North’s research interests include the molecular basis of inherited muscle disorders, genes that influence skeletal muscle function, and new therapies for muscular dystrophy and neurofibromatosis type 1.

Dr Antonio Penna Dr Penna was appointed to the position of Chief Executive of The Children’s Hospital at Westmead following Professor Kim Oates’ retirement in 2006. For one year prior to this he was Director of Clinical Services – Medical. Before joining The Children’s Hospital at Westmead, Dr Penna was

the Director of Medical Services at Royal North Shore Hospital, a position held since 1997. He completed his paediatric training at the Adelaide Children’s Hospital and was an NHMRC Postgraduate Fellow at the University of Melbourne, where he completed his doctorate in pharmacokinetics. In 1992 he became Clinical Superintendent in the Department of Paediatrics at Westmead Hospital, where he was subsequently promoted through a range of administration positions while maintaining a clinical role.

Mr Daniel Petre AO

Mr Petre worked in the technology industry in Australia for more than 20 years. Prior to founding Netus, a technology investment company, he spent nine years at Microsoft including three years as Managing Director, Australia. He went on to found Ecorp, a subsidiary of Publishing and Broadcasting

Limited (PBL), which became Australia’s leading internet company. Mr Petre has made an extensive contribution to the not-for-profit sector, with positions held on the Area Health Advisory Council for The Children’s Hospital at Westmead, the Advisory Board of HealthInsite and The University of New South Wales Foundation, among many others. Mr Petre and wife Carolyn set up the Petre Foundation in 2000, which has funded research chairs both at The Children’s Hospital at Westmead and the Garvan Institute as well as a scholarship for university medallists at the University of New South Wales.

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14 Finance

Finances

Operating Statement for the year ended 30 June 2009 (unaudited)1

2008/09$000's

2007/08$000's

Revenue from all sourcesResearch Grants 12,344 5,822

Donations and other Contributions 8,827 8,803

Infrastructure Support2 1,894 2,108

Block Funding3 1,077 834

Investment Income 432 (494)

TOTAL REVENUE 24,574 17,073

Expenditure on research operationsEmployee Related Expenditure 13,459 11,553

Goods and Services 6,313 5,905

Repair, Maintenance, and Renewals 465 781

Depreciation 897 995

TOTAL EXPENDITURE 21,134 19,234

RETAINED SURPLUS / (DEFICIT) 3,440 (2,161)

Resource Allocation ($000’s)

Revenue Expenditure

50%Research

Grants$12,345

36%Donations andContributions

$8,827

8% Infrastructure Support $1,894

4% Block Funding $1,077

2% Investment Income $432

2% InvestmentIncome $432

4% Block Funding $1,077

8% Infrastructure Support $1,894

50% Research

Grants$12,344

36% Donations and Contributions

$8,827

30%Goods andServices$6,313

64%EmployeeRelated$13,459

4% Depreciation $8972% Repairs, Maintenanceand Renewals $465

4% Depreciation $8972% Repairs, Maintenance and Renewals $465

64% Employee Related$13,459

30% Goods and

Services$6,313

Depreciation

Repairs, Maintentance and Renewals

Goods and Services

Employee Related

Notes 1. Kids Research Institute is the research division of The Children’s Hospital at Westmead. Separate financial statements are not audited for the Kids Research Institute. The consolidated annual audited financial statements of The Children’s Hospital at Westmead are available on request or you can visit our website at www.chw.edu.au 2. Infrastructure support has been provided by The Children’s Hospital at Westmead. 3. Block funding has been provided by NSW Department of

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15 Finance

Finances

Balance Sheet as at 30 June 2009 (unaudited)1

2008/09$000's

2007/08$000's

Current AssetsCash at Bank and Investments 25,200 27,572

Non-Current AssetsInvestments 7,108 -

Buildings4 18,090 17,834

Plant and Equipment 1,963 3,258

NET ASSETS 52,361 48,664

Represented byAccumulated Funds 48,664 50,825

Revaluation Reserve 257 -

Retained Surplus / (Deficit) 3,440 (2,161)

RETAINED EQUITY 52,361 48,664

Resource Allocation ($000’s)

Assets

47%Current

Cash andInvestments

$25,200

14%Non-CurrentInvestments

$7,108

35% Buildings$18,090

4% Plant and Equipment $1,963

4% Plant and Equipment $1,963

35% Buildings$18,090

47%Current

Cash and Investments

$25,200

14% Non-Current Investments

$7,108

Health to the National Centre for Immunisation Research and Surveillance. 4. The main building of Kids Research Institute is owned by The Children’s Hospital at Westmead. The estimated value of buildings recognised above is based on the floor space occupied by Kids Research Institute.

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Your child: the centre of your universe, the centre of our research

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18 Annual Report 2008-2009

Group Leader: Professor Kathryn North, Department Head

Stream 1 - Neurosciences and Mental Health

Institute for Neuroscience and Muscle ResearchMaking the lives of children better through advances in muscle and nerve research

The Neurosciences and Mental Health stream researches the effect of the brain and muscle on children’s health and well-being. Its activities range from research into neurological diseases such as muscular dystrophy and neuromuscular disorders to developmental cognitive neuropsychology research. This involves understanding how brains work in children with varying conditions from brain tumours to sleep disorders and dyslexia, and improving the interface between health and education in children with a range of neurological conditions. Other research involves improving the understanding of the effectiveness of interventions in children and adolescents with eating disorders.

Overview The Institute for Neuroscience and Muscle Research (INMR) investigates causes, consequences and therapies for specific inherited and acquired neurological diseases such as the muscular dystrophies, peripheral neuropathies, neuroinflammatory disorders such as multiple sclerosis and neurofibromatosis type 1.

The INMR is a multi disciplinary team involving over 40 clinicians, research scientists, genetic counsellors, physiotherapists, occupational therapists and psychologists, who care for more than 1800 patients and their families.

Research is directly relevant to patients. Clinical trials can be immediately incorporated into treatments and therapies and the diagnostic service provides families with accurate diagnosis and disease specific treatment. The integration of clinical and laboratory research and the clinical interface with patients within a hospital setting makes the INMR unique. Its research and the clinical care it provides patients are recognised for their excellence nationally and internationally.

Research AchievementsOne major focus of the INMR’s research program is to improve treatment and diagnosis of neuromuscular disorders. More than 60 different types of neuromuscular diseases have been identified and most are incurable.

For the first time in Australia, children affected by Duchenne muscular dystrophy have access to medication that has transformed their lives through participation in a clinical trial led by the INMR. This treatment has enabled patients who would normally

have been confined to a wheelchair to run, jump and kick a ball.

Other clinical trials are underway which give patients access to experimental medication for the first time. These include an international multi-centre trial funded by the US government which focuses on the treatment of cognitive deficits in Neurofibromatosis type 1.

Recently published work on NMDA-R encephalitis in children will improve identification of this treatable yet disabling condition. The INMR has recently identified an important autoimmune response against myelin oligodendrocyte glycoprotein in children with acute brain demyelination. This finding has potential implications for both childhood and adult multiple sclerosis, and will hopefully define tailored therapy in the future.

Another study involves a particular group of molecules called aquaporins, which play a significant role in hydrocephalus. The INMR has demonstrated that absence of aquaporin 1 can reduce the effects of hydrocephalus in mice, and aims to translate this finding into a new medical treatment for patients.

This treatment has enabled patients who would normally have been confined to a wheelchair to run, jump and kick a ball

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19 Annual Report 2008-2009

Group Leaders: Dr Sloane Madden, Co-Director, Associate Professor Michael Kohn, Co-Director

Group Leader: Dr Belinda Barton, Department Head

Department of Adolescent Medicine and Eating DisordersDriving innovation in the treatment of eating disorders

Children’s Hospital Education Research InstituteImproving learning in sick children

OverviewThe Eating Disorder Service at The Children’s Hospital at Westmead is the largest child and adolescent eating disorder service in NSW and an Australian leader in the provision of care for this group.

Hundreds of children throughout NSW and beyond receive care and support from the eating disorder team through its inpatient and outpatient treatment services and its educational, mentoring and supervision projects. Key to its success has been a strong clinical research focus driving innovation in the treatment of children and their families with this debilitating condition.

The Service’s current research interests include family treatment in eating disorders; early onset eating disorders in children under the age of 12; neuroimaging in eating disorders; refeeding and body composition; neurophysiology in eating disorders, and bone changes in eating disorders.

Overview The Children’s Hospital Education Research Institute (CHERI) conducts research which promotes children’s health and well-being through improvements in the interface between health and education.

CHERI’s research program focuses on establishing the cognitive and psychosocial profile of children with medical, developmental and/or psychological conditions such as neurofibromatosis type 1 and velocardiofacial syndrome.

Research AchievementsCHERI’s research has centred on characterising the cognitive profile of children with a clinical condition who are also experiencing learning difficulties. In conjunction with the Institute for Neuroscience and Muscle Research, CHERI is involved in an international clinical trial to determine whether lovastatin, a cholesterol lowering medication, reverses learning problems in children with neurofibromatosis type 1 (NF1).

Research AchievementsIn 2007 The Children’s Hospital at Westmead eating disorder research team received an NHMRC grant for more than $500,000 to look at the optimal role of hospital in treating children with eating disorders. This grant, one of only two such grants for eating disorder research in Australia, compares the efficacy of short versus long hospital stays for weight restoration. When completed this randomised controlled trial will be the largest trial of hospital care in eating disorders.

In other research, the team is looking at the role of family-based treatment in reducing hospital admission rates. It is also conducting ongoing neuroimaging research looking for unique neurophysiological markers of anorexia nervosa.

An important epidemiological study on early-onset eating disorders in Australian children, published in 2009, received widespread public attention when it found an increase in advanced eating disorders in children as young as five, one in four of them boys.

It is also following a cohort of young children with NF1 from infancy to school age. The results of this study will allow for the early identification of children with NF1 at risk of academic failure and subsequent early intervention. This is the first longitudinal study of young children with NF1.

CHERI is conducting a study that aims to identify the cognitive and psychosocial deficits that are specific to children with velocardiofacial syndrome by comparing their profile to an intellectually-matched control group. Other current studies focus on improving children’s well-being, CHERI is examining the role of social support from parents, teachers and peers on the health related quality of life of children newly diagnosed with cancer.

Other work involves the examination of the vocational development of adolescents with cystic fibrosis and a national study to identify the support needs of full time workers who care for a child with a chronic illness. Results of this study will be used to make recommendations to policy makers.

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Group Leader: Associate Professor Pam Joy, Department Head

Developmental Cognitive Neuropsychology Research UnitUnderstanding the neuropsychology of sick children

Overview The Developmental Cognitive Neuropsychology (DeCog) Research Unit is an internationally-recognised developmental neuropsychology research facility.

Its research activity focuses on investigating neuropsychological outcomes and monitoring neuropsychological status in children with developmental or acquired disorders. Knowledge gained from these studies is likely to have a significant impact on medical and cognitive outcomes in children with quite varying conditions, including children with acquired cerebellar disease, brain tumour, diabetes and sleep disorders.

DeCog is the only developmental cognitive neuropsychological research unit in Australia. It conducts cognitive neuropsychological studies in children with disorders such as dyslexia or face processing deficits, using theoretical models to explain cognitive processes and to aid understanding

of developmental disorders. These studies focus particularly on the development of treatment and intervention programs to improve outcomes in children with a range of medical and developmental conditions.

Research AchievementsDeCog continues to provide numerous ongoing research projects and publications.

At the core of its research, cognitive studies build on existing theories and models to improve understanding of developmental cognitive neuropsychology disorders. Current research areas include developmental or acquired social processing in children impairment in visual processing skills and the nature of impairment in children with dyslexia who are unable to process individual letters.

Research into neuropsychological outcomes and ongoing collaborations have continued this year. These studies form a special research focus regarding the neurological, behavioural and psychological consequences of traumatic brain injuries, tumours, diseases and syndromes. Studies include acquired cerebellar disease in childhood; recovery of language skills in the Landau-Kleffner Syndrome, and neuropsychological outcome in long term survivors of low grade brain tumours in infancy and childhood.

DeCog also conducts a range of projects which focus on monitoring the effectiveness of medical interventions in relation to neuropsychological functioning. For example, research is looking at the long term effects of hypoglycaemia on the executive functioning of children with type 1 diabetes. Another study is assessing changes in cognition, mood and behaviour in children with type 1 diabetes starting insulin pump therapy.

A Reading and Spelling Disorders Clinic was established this year, based on principles derived from empirical research completed at DeCog. The first such initiative in NSW, this clinic offers assessment and treatment for children and adolescents with reading and spelling disorders. The clinic provides evidence-based remediation programs to the most severely affected children.

Knowledge gained from these studies is likely to have a significant impact on medical and cognitive outcomes in children with quite varying conditions

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21 Annual Report 2008-2009 | Stream 1 - Neurosciences and mental health | Stream 1 - Neurosciences and mental health

Snapshot: Eating Disorder Service at the Departments of Psychological Medicine and Adolescent Medicine

“Parents are the most motivated to help children get over their illness.” - Dr Sloane Madden

Family therapy helps children eat againEating disorders are showing up in younger and younger children. A study conducted by the Eating Disorder Service at the Kids Research Institute found children as young as five were developing anorexia nervosa, with many of them becoming so sick they needed to be hospitalised.

Children with eating disorders are subject to many serious medical complications from their illness including growth retardation, damaged bone health, impairments in brain health and potentially life threatening complications of low heart rate, low blood pressure and low temperature.

The eating disorder research team has recently received an NHMRC grant to conduct Australia’s largest eating disorders trial - the first randomised controlled trial in the world - to look at inpatient care in children and adolescents with an eating disorder. All participants in the trial are given family treatment after hospital.

“We have moved away from thinking of the family being responsible for causing a child’s eating disorder to it being a major way of treating it,” says Department Head Dr Sloane

Dr Sloane Madden, Co-Director, Eating Disorder Service

Madden. “Parents are the most motivated to help children get over their illness.”

Kim Jones has spent much of this year attending weekly counselling sessions at The Children’s Hospital at Westmead after her 12 year old daughter very suddenly developed an eating disorder after she started high school.

She had lost 12 kg and her heart rate was just 40 beats per minute when she was admitted to be re-fed during a three-week hospital stay in April.

Now Kim has helped her daughter well on the road to recovery. She can eat unsupervised, has reached her target weight and is gaining confidence.

“She was so frightened by what happened but now she feels about 50% in control,” says Kim. “We’re just at the beginning of a long journey, but I’ll be with her all the way.”

*Name has been changed

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Group Leader: Associate Professor David Little, Department Head

Overview The Orthopaedic Research and Biotechnology Unit (ORB) aims to advance orthopaedic care through an improved understanding of bone diseases, bone healing and pharmaceutical therapies. Its research approach is highly translational, bridging the gap from bench to bedside.

ORB encompasses 11 dedicated research staff working on a wide range of translational projects. It has had significant academic and grant success, including five prestigious national NHMRC grants with a combined value of over $1.5 million as well as other small grants and industry collaborations. ORB

has been credited with 37 peer-review publications in the past five years, many in high impact bone and orthopaedic journals.

Research AchievementsORB pioneered the use of bisphosphonates in paediatric medicine and this work is now directly helping children within the Hospital. Associate Professor Little’s bisphosphonate research is internationally recognised. This work has led to the

Orthopaedic Research and Biotechnology UnitWorking hard to help kids play

Stream 2 - Tissue Engineering and Bone Repair

This stream is advancing knowledge of how the body heals itself, which is translated into better treatments to promote healing. Orthopaedic and biotechnology research is working to understand bone diseases and bone repair. A wound healing laboratory is investigating what happens at a cellular level when wounds heal especially after suffering a burns injury.

Research is highly translational, bridging the gap from bench to bedside

Staff profile: Dr Joshua Burns, NHMRC Clinical Research Fellow, Institute for Neuroscience and Muscle Research

Dr Joshua Burns is a podiatrist who has spent the last three years at the Institute for Neuroscience and Muscle Research studying foot pain and disability in children with inherited nerve disease.

His work involves improving the treatment of neurological disorders such as Charcot-Marie-Tooth disease (CMT), which is characterised by foot deformity and walking difficulty, and Duchenne muscular dystrophy, for which there is no cure.

“Understanding and helping children with progressive neuromuscular disorders is extremely interesting and I enjoy the challenge of finding the best measures to assess disease severity and the most effective therapies to help improve quality of life,” Dr Burns says.

So passionate is he about his work that this year he cycled from Sydney to Melbourne to increase awareness about Duchenne muscular dystrophy.

He plays a leading role in advocacy and raising awareness for CMT patients.

Three years out from his PhD, Dr Burns has already established an international reputation for his research into CMT. Among a string of awards is the NSW Young Tall Poppy Science Award and a Fulbright Scholarship, which this year has taken him to the USA to work on a CMT paediatric scoring system.

His future research directions will involve the role of Vitamin C therapy in CMT, foot and ankle training in CMT and a trial of oral curcumin in Déjerine-Sottas disease.

“Through a significant expansion of my postdoctoral studies, I aim to promote ongoing collaboration between Australian and international sites to enhance our ability to translate basic biomedical research into evidenced-based clinical therapies for children with these diseases,” he says.

A tall poppy strides out for research

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development of one of the ORB’s main research themes - optimisation of the anabolic (bone-forming) and catabolic (bone-resorbing) responses. It has had success in combining local anabolic treatment with systemic bisphosphonate therapy. The Unit is continuing to explore this drug combination experimentally as well as applying it to clinical situations.

NF1 is a common genetic disease, affecting one in 3,000 children, that often manifests as tumour formation. These children can also develop severe orthopaedic problems, including scoliosis (curvature of the spine) and a congenital tibial dysplasia. Orthopaedic surgery on NF1 children has a poor prognosis due to underlying problems with bone healing. ORB has been one of the leading contributors in the international push to define the mechanism of

these bone defects, and has published studies that indicate that dual problems in bone anabolism and bone catabolism may both contribute to the NF1 bone phenotype.

In a recent world first, ORB has unequivocally demonstrated that cells from the surrounding muscle are critical in serious skeletal injury. These cells make a key contribution to bone formation and repair and researchers are already learning how to manipulate these findings to provide improved outcomes for children. This project may also result in new cell-based therapies for bone repair that utilise muscle cells.

Other work involves promoting fracture repair with the use of novel pharmaceutical agents which can bolster the bone forming anabolic response, while keeping the bone resorbing catabolic response to a minimum.

Group Leaders: Associate Professor Andrew Holland, Director, Children’s Hospital Burns Research InstituteDr John Harvey, Department Head, Burns Unit, Dr Rachael Murray, Head, Wound Healing Laboratory

Overview The Children’s Hospital Burns Research Institute (CHBRI) was formed in 2005 to build upon the strong reputation held by the Burns Unit at The Children’s Hospital at Westmead for clinical excellence in the treatment of children with burns injury.

CHBRI seeks to build upon this established clinical track record and introduce an exciting new element - laboratory-based burns research.

Within CHBRI, the Wound Healing Laboratory is researching how the skin heals at an intra-cellular level.

Research AchievementsCHBRI pioneered the use of a new technique of Laser Doppler Imaging in predicting burn wound outcome in children. A laser light is used to determine flow

beneath the wound and thus to predict its depth and inform treatment decisions. Recently, new equipment has been devised which enables the process to be completed in five seconds rather than three minutes. Studies into the usefulness of this technique are continuing.

Other work is looking at the role of fibrocyte cells in hypertrophic (thick, raised) scarring and the molecular biological process which regulates burn wound healing.

In other areas, CHBRI has confirmed the role of running water as the optimal burns first aid and treatment in children; determined the optimal time to graft a scald burn, and looked at long term clinical outcomes to determine the influence of time of healing on the development of a hypertrophic scar.

Children’s Hospital Burns Research Institute (CHBRI) Improving the treatment of burns and wounds in children

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24 Annual Report 2008-2009 | Stream 2 - Tissue Engineering and Repair

Snapshot: Orthopaedic Research and Biotechnology Unit

“The doctors have helped nature do its job.” – Carol Strebel

Osteoporosis drug puts Cameron back on his feet

Cameron Strebel, 11, dislocated his hip in his friend’s front yard two years ago. Undiagnosed for several days, by the time he arrived at The Children’s Hospital at Westmead he was suffering from osteonecrosis of the femoral head – the bone had died.

There are no effective treatments for osteonecrosis. Several years ago, Cameron would have faced a life of pain and disability. But research conducted by the Orthopaedic Research and Biotechnology Unit has led to a new treatment which has allowed his hip to grow back by itself.

Associate Professor David Little’s work into therapy using osteoporosis drugs bisphosphonates is internationally recognised. The Orthopaedic Research and Biotechnology Unit was also the first to show that zoledronic acid therapy led to significant preservation of the structure and shape of the femoral head in rats.

Without treatment, the femoral head would completely collapse. But in a two-year follow up of the first 17 patients to be treated with the new therapies, over half had completely round femoral heads and another third had oval shaped femoral heads – in other words, the treatment had preserved the children’s ability to walk.

Cameron was confined to a wheelchair for 18 months after his accident and has undergone several rounds of surgery to further improve his hip function.

This surgery combined with the new drug therapy now means he is able to walk, jump and play sport again and more importantly, his newly grown hip will last him well into his adult life.

“The doctors here have helped nature do its job and this way the hip will grow with him,” says Cameron’s mother Carol Strebel.

“I cannot speak highly enough of Associate Professor Little’s team.”

Further research is required to further improve outcomes, but this research and its application illustrate the potential benefits of new treatments for this potentially devastating problem.

Associate Professor David Little, Head, Orthopaedic Research and Biotechnology Unit

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Osteoporosis drug puts Cameron back on his feet

Staff profile: Renjing Liu, PhD Student, Orthopaedic Research and Biotechnology Unit

When orthopaedic surgeons repair a shattered bone, they sometimes wrap muscle around it. It has long been believed this increases blood supply which promotes the bone’s healing.

However, by tracking the cells involved in the process of bone repair, PhD student Renjing Liu has discovered that muscle stem cells can actually turn into bone and make significant contributions to the healing process.

“Current understanding of which cells contribute to bone formation is lacking, and if we don’t know which cells participate we can’t really have the right treatments,” she says.

One of the problems has been tracking cells in the human body. The Orthopaedic Research and Biotechnology Unit has used genetically modified mice which enable muscle stem cells to be

specifically and permanently labelled, meaning they can be easily tracked.

The work has confirmed clinical and experimental observations that the body uses muscle cells as a backup system to make new bone when the cells that are normally involved in this process are absent.

The findings will have direct implications for humans. Understanding the role of muscle stem cells in bone repair will lead to the development of therapies that will enhance their ability to contribute to the healing process.

Some of Ms Liu’s findings have already been published and presented internationally and she is now looking forward to conducting further research which will hopefully translate her findings into clinical applications that will improve patient care.

How muscle helps repair broken bones

Stream 3 - Cancer Biology

The Children’s Hospital at Westmead treats about 120 children with cancer every year. Oncology researchers undertake basic, clinical and translational cancer research, particularly into child and adolescent tumours which have a poor patient prognosis. Researchers are investigating the role of gene therapy in treating cancers and are developing molecularly-targeted therapies. A Tumour Bank holds more than 20,000 samples of childhood tumours for use in research in Australia and overseas.

Oncology Research UnitBasic, clinical and translational cancer research

Group Leader: Associate Professor Jennifer Byrne, Acting Department Head

Research programs aim to increase understanding of the molecular and cellular basis of childhood and other cancers and to develop new cancer treatments using gene therapy and molecularly-targeted approaches. The Unit also aims to facilitate and promote research into childhood cancer through the provision of high quality clinical specimens, laboratory research platforms and research expertise.

Research AchievementsThis year, researchers in the Focal Adhesion Biology Group made a major advance in understanding how cell migration is regulated in cancer. Members of the group were part of an international, cross-disciplinary

Overview The Oncology Research Unit is the research-dedicated arm of the clinical Oncology Department. It undertakes basic, clinical and translational cancer research.

The Unit carries out research into a number of cancer types, with a focus on the solid tumours of childhood and adolescence that have a poor patient prognosis such as neuroblastoma, brain tumours and childhood sarcomas.

As experimental models are not available for all cancer types, researchers within the unit also use models of more common cancers to discover critical mechanisms in tumour progression. These discoveries can then be applied to rarer childhood cancers.

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Staff profile:Dr Belinda Kramer, Oncology Research Unit

The very large doses of chemotherapy required to kill some kinds of brain tumours also destroy healthy cells, particularly in the bone marrow. This means the drugs are too toxic for young patients and their tumours may not be completely eradicated.

But an Oncology Research Unit project will soon make that a thing of the past. Laboratory Project Leader Dr Belinda Kramer has been working for 10 years to implement a gene therapy strategy that makes bone marrow stem cells resistant to the damaging effects of chemotherapy.

The stem cells are removed from the patient, a modified gene inserted into them to protect them from the chemotherapy drugs and they are then transplanted back into the patient.

Dr Kramer and her laboratory team have generated a vector under cleanroom conditions that will expose

the cells to the modified genes. It is awaiting approval by the Therapeutic Goods Authority and a Phase 1 trial of the therapy will begin in 2010.

“This will be the first cancer gene therapy trial targeting bone marrow stem cells in Australia,” Dr Kramer says.

“We know these cancers can be sensitive to the drugs if we give more of the chemotherapy but at the moment we just can’t do it. For children whose tumour has relapsed and who can’t ordinarily be given higher doses, this strategy could both reduce chemotherapy side effects and more effectively treat their tumour.”

If the trial is successful, the drug resistance strategy will have applications for many diseases other than cancer.

Making cells invincible against chemotherapy

collaboration which published the first reported protein structure of the C-terminus of the pro-metastastic protein NEDD9. This new knowledge will enable the future design of molecules to inhibit the function of this protein and thus slow cancer metastasis.

Funding through the Clinical Research Fellowship (Cancer Institute of NSW) has for the first time allowed the Neuroblastoma Research Group to identify “ALT” (alternative lengthening of telomeres) as a distinct mechanism by which neuroblastoma cancer cells maintain the ends of chromosomes and thereby allow cells to grow indefinitely. This knowledge will enhance the understanding of the cause of neuroblastoma, which is one of the most common childhood cancers.

In 2008, the Cancer Gene Therapy Group made significant progress in the initiation of a Cancer Gene

Therapy Clinical Trial, with the successful production of the gene transfer reagent (vector) required for use in the trial. This vector was produced under cleanroom conditions at The Children’s Hospital Westmead within the Gene and Cell Medicine Facility and has been qualified for clinical use, meeting standards for testing by two independent laboratories. The trial, once approved, will provide an alternative therapy for patients with poor prognosis tumours as it will allow high doses of chemotherapy to be administered without increasing the side effects that would normally accompany this treatment.

Ms Cuc Bach, PhD student in the Focal Adhesion Biology group, was awarded a NSW Cancer Institute Scholar Award for her PhD studies.

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27 Annual Report 2008-2009 | Stream 3 - Cancer Biology

Snapshot: Tumour Bank

“We are gaining new knowledge from these samples to develop and improve our understanding of cancer.” – Dr Dan Catchpoole

Every year, more than 120 children with cancer are treated at The Children’s Hospital at Westmead. By studying their tumours, researchers are able to better understand paediatric cancers and translate their findings into new treatments for children worldwide.

To do this, researchers need samples of cancers to study. The Tumour Bank was established 10 years ago as the first facility in Australia dealing exclusively with paediatric cancer in Australia. It now stores 20,000 tissue specimens which have been donated by more than 3,000 patients.

Small samples are collected with parents’ permission, de-identified and used for research both in the Kids Research Institute, elsewhere in Australia, and internationally.

The Head of the Tumour Bank, Professor Dan Catchpoole, says understanding the unique genetic coding of tumours enables doctors to more precisely diagnose cancers - a process which currently relies largely on clinical skill - and also to develop more targeted therapies.

“Of all the cancers we see in our Hospital every year, we get various types and sub types of tumours and lots of rarities,” Professor Catchpoole says.

“We have been able to collect tissue over time and build up a decent cohort of samples. It enables researchers in Australia and overseas to collect enough material to obtain statistical strength from their studies.”

New technologies are enabling the Tumour Bank to dissect the tiny tumour samples into smaller and smaller sections so that as much information as possible can be gained from them.

It has recently received a grant to establish a “virtual microscope” which will make viewing high resolution microscopic images of tissue samples possible via the internet. These images are securely housed on a server and are available to approved researchers.

“New technology has allowed better and more novel approaches to our research,” says Professor Catchpoole.

“Our whole goal is translational - gaining new knowledge from these samples to develop and improve our understanding of cancer and making it applicable to a real world situation.”

Dr Dan Catchpoole, Head, Tumour Bank

Unlocking the secrets of childhood cancers

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28 Annual Report 2008-2009

Group Leader: Professor John Christodoulou, Department Head

Human Genome Research Program (Research arm of the Western Sydney Genetics Program)Understanding the biology of genetic disorders to develop better treatment for children

The Genetic Skeletal Disorders and Lysosomal Research Group researches rare inherited disorders which result in a large number of patients requiring rehabilitation through the Hospital’s busy clinics. The Group has recently prepared an updated clinical and genetic classification of the Osteogenesis Imperfecta syndromes and has pioneered the bisphosphonate treatment of these disorders in Australia. This work will benefit all children with osteoporosis as it has resulted in the development of normal ranges for bone density in children, seen the trial and evaluation of multiple forms of bisphosphonates and enabled the findings to be applied to a wider range of skeletal disorders of childhood.

The NSW Newborn Screening Program screens all newborns in NSW and the ACT for the common treatable inborn errors of metabolism. Its major research focus this year has been DNA mutational analysis of dried blood spot samples with a particular focus on Fragile-X Syndrome.

The Eye and Developmental Genetics Research Group works to identify and characterise novel disease genes in the eye and other developmental disorders. Major recent achievements involve characterisation of a critical signalling pathway in eye development, as well as advances in the understanding of corneal stem cell function and the identification of a novel familial mutation in the eye development gene SOX2.

Overview The Western Sydney Genetics Program and its research arm, the Human Genome Research Program, are devoted to understanding the basis of a number of genetic disorders from a biological perspective and translating these research findings into clinical practice.

The Program consists of a number of research groups, each with its own group leader, which conduct research in diverse areas: the Genetic Metabolic Disorders Research Unit, the NSW Biochemical Genetics Service, the Department of Clinical Genetics, the NSW Newborn Screening Program, the Eye and Developmental Genetics Research Group, and the Genetic Skeletal Disorders and Lysosomal Research Group.

Activities range from basic laboratory research through to applied or translational research and clinical studies. Staff are in the unique position of often working directly with families affected by some devastating conditions. Through this clinical interface they are able to offer better diagnosis, prognosis and novel treatment options as a result of their genetic research.

Research AchievementsThe Genetic Metabolic Disorders Research Unit, which incorporates the NSW Centre for Rett Syndrome Research, studies a number of genetic disorders that in particular affect brain development and function. The Unit has recently discovered the gene responsible for Arts Syndrome, allowing the implementation of a new therapy. It has also discovered a gene causing a mitochondrial respiratory chain disorder, a genetic modifier in Rett syndrome and has demonstrated that environmental enrichment is of benefit in a mouse model of Rett syndrome.

Research into genetics and the human genome aims to understand the biology of genetic disorders, including rare disorders such as Rett Syndrome and Arts Syndrome. All newborns in NSW and the ACT are screened by the NSW Newborn Screening Program for errors of the metabolism. Other research areas involve eye and developmental genetics, genetic skeletal disorders and lysosomes. Another important area is gene therapy, which aims to treat genetic conditions in children by replacing faulty genes with healthy ones.

Stream 4 - Genetics, Gene Therapy and Genomics

Through their clinical interface, staff are able to offer better diagnosis, prognosis and novel treatment options as a result of their genetic research

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29 Annual Report 2008-2009 | Stream 4 - Genetics, Gene Therapy and Gnomics

Snapshot: Genetic Metabolic Disorders Research

“We are acutely aware of the important factors for patients and their families, and it is this that often influences our research directions.” - Professor John Christodoulou

Gene discovery changes brothers’ livesThomas and Bradley Farrell have been regular visitors to The Children’s Hospital at Westmead their whole lives. The boys were both born profoundly deaf, their progressive muscle weakness means they have never walked and they are both gradually losing their sight.

Every six months the boys would catch colds which rapidly progressed to pneumonia and total paralysis of the breathing muscles, meaning they would spend weeks on end in intensive care.

Initially no-one could give the family an accurate diagnosis, until three years ago when doctors at The Children’s Hospital at Westmead discovered the boys had Arts syndrome, a very rare genetic disorder that affects boys. The Farrell brothers are believed to be the only children in the world with the syndrome.

Recently, researchers from the Genetic Metabolic Disorders Research Unit, one of the research arms of the Western Sydney Genetics Program, working in collaboration with teams in the Netherlands and Brisbane, made another significant breakthrough when they identified the gene responsible for Arts syndrome.

They could then conduct genetic analysis to identify the mistake in the gene that was responsible for the syndrome. That has led to a therapy for the boys.

“We looked at the biology of the biochemical pathway in which the gene was believed to participate and were able to propose an existing drug, S-adenosylmethionine (SAM), as a

possible treatment,” says Director of the Program, Professor John Christodoulou.

The therapy worked. Since it started two years ago, the boys have only spent 10 days in Hospital compared to 260 days between them previously.

There have also been improvements in their stamina and their ability to learn, while the deterioration in Thomas’s muscle function has slowed. “Now they can enjoy their lives,” says their mother Sharen.

Professor Christodoulou’s research focuses on genetic disorders which affect brain development and function, with the aim of better understanding the biology of these disorders and ultimately developing better, more targeted therapies. He has been widely recognised for his contribution to the field of human genetics.

His work provides the opportunity for better diagnosis and prognosis. It also has potential for improved understanding of the biology of these often complex disorders and enables more accurate genetic counselling to families.

“Being at the clinical interface, we are acutely aware of the important factors for patients and their families and it is this that often influences our research directions,” says Professor Christodoulou.

Professor John Christodoulou, Director, Western Sydney Genetics Program

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The key challenge is to safely repair or replace faulty genes with healthy ones in a sufficient number of cells to achieve therapeutic benefit

Gene Therapy Research UnitThe answer is in our genes

Group Leader: Professor Ian Alexander, Department Head

Overview The Gene Therapy Research Unit, a joint initiative with the Children’s Medical Research Institute, focuses on the development of novel gene-based strategies for the treatment of genetic conditions affecting children. This is a tremendously exciting but challenging area requiring both laboratory and clinical research expertise.

The key challenge is to safely repair or replace faulty genes with healthy ones in a sufficient number of cells to achieve therapeutic benefit.

The approach involves the use of cutting-edge gene delivery technologies (vectors) derived from viruses and begins with genetic repair of isolated cells growing in cell culture followed by treatment of disease in small animals such as mice.

The liver and bone marrow are two particularly promising targets for gene therapy as many childhood genetic diseases involve these tissues.

Research AchievementsExceptional progress is being made in all areas, but of particular interest is the Unit’s work on metabolic liver disease, which is on the cusp of being translated from the laboratory into treatment for children with the disease.

The Unit has previously reported success in curing mice with Ornithine Transcarbamylase (OTC) deficiency using gene therapy. The current challenge is to ensure that the gene delivery strategy that the Unit has devised will be safe and effective in humans. It has developed a research pathway to test the delivery of the human OTC gene to human liver cells and established the necessary collaborative relationships needed to reach this goal. This project is well on the way to generating the data necessary to justify and underpin a human clinical trial proposal.

Institute of Endocrinology and DiabetesWorking to prevent diabetes and osteoporosis in children

Group Leader: Associate Professor Chris Cowell, Department Head

Overview The Institute of Endocrinology and Diabetes is one of the world’s leading clinical paediatric endocrinology research centres. Its research staff leads basic and epidemiological studies into the causes of childhood diabetes and their complications and conducts clinical trials that aim to prevent or cure diabetes and osteoporosis in children.

The central theme of research is prevention. The Institute’s ultimate objectives are to prevent the onset of type 1 and type 2 diabetes in children, to prevent the

development of diabetes complications and to prevent the morbidity, such as bone pain and fractures, in children with primary and secondary osteopenia.

Research AchievementsThe Institute is internationally recognised for its expertise in type 1 diabetes research into epidemiology and complications. This year it has had publications in the British Medical Journal, Diabetes Care and Diabetic Medicine. The Institute is co-investigator in several international studies into the prevention of

The Obesity, Metabolism and Nutrition stream aims to prevent and treat complications resulting from disorders of the metabolism such as diabetes, osteoporosis and obesity. It incorporates one of the world’s leading clinical paediatric endocrinology research centres as well as an Obesity Research Group which focuses on the burgeoning problem of child and adolescent obesity. Other work looks at the efficacy of exercise in improving metabolism, muscle and bone strength in children with a range of chronic conditions.

Stream 5 - Obesity, Metabolism and Nutrition

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Obesity is a major public health problem affecting a growing number of children

Overview The Obesity Research Group and its collaborators undertake research that looks at the causes and consequences of child and adolescent obesity. Obesity is a major public health problem affecting a growing number of children from many countries, including Australia, which is associated with a range of medical and psychosocial complications.

The Group’s work concentrates on the metabolic complications of obesity in children and the factors influencing the development of obesity which can potentially be modified. It is also studying the types of treatment programs that can help children and young people already affected by the condition. Other work investigates how the problem of obesity can be prevented from occurring in the first place.

Research AchievementsPaediatric physiotherapist and PhD student, Genevieve Dwyer, developed and validated a tool for measuring physical activity in preschool-aged children, the Pre-PAQ® (Preschool-aged children’s Physical Activity Questionnaire). Previously there were no simple tools

available for professionals to use to measure physical activity levels in large groups of three to five year olds.

The Obesity Research Group undertook a range of studies looking at options for treating children and adolescents affected by obesity. The Obesity Research Group was one of two sites conducting the innovative PEACH trial, which looked at the effect of parenting interventions on weight outcomes for pre-adolescent children. The Loozit® study is a community-based weight management program for obese adolescents which recent research has shown to be effective in decreasing waist circumference, improving participants’ lipid profile and boosting their self esteem.

Additional work has included participation in major systematic reviews of obesity treatment in children and adolescents and qualitative studies of adolescent-parental interactions around weight issues. This suite of studies includes some of the first lower-cost obesity treatment interventions for use in community settings.

The Obesity Research Group conducted a major Cochrane review on the treatment of child and adolescent obesity and held a world first case discussion of the child protection issues associated with severe childhood obesity.

Group Leader: Professor Louise Baur, Department Head

Obesity Research GroupManaging and preventing obesity in Australia’s youth

The central theme of research is prevention of diabetes in children, diabetes complications, and morbidity

complications, for example using diet modification (the AdDit study), reducing the incidence of diabetes in the genetically at risk (the TRIGR study) and the use of intranasal insulin in the national diabetes prevention study. Basic studies investigating the viral aetiology of type 1 diabetes are demonstrating effects of enteroviruses on beta cells.

With about 11% of all new cases of diabetes diagnosed in adolescents associated with obesity, and many more adolescents developing metabolic complications of obesity, the Institute has commenced two randomised controlled trials to try to reverse the metabolic abnormalities and prevent development of diabetes. RESIST is comparing the effects of diets that vary in their carbohydrate and protein content

on insulin sensitivity in combination with an intense exercise program. VIBRATE is examining the effect of whole body vibration on insulin sensitivity.

Vitamin D deficiency rickets is a major re-emerging problem is Australia, particularly among recent migrants, children with dark skin colour and girls who are covered for cultural or religious reasons. The Institute has commenced a study to assess the use of high dose (Stoss) vitamin D therapy for rickets that should result in change to routine clinical practice.

Whole body vibration training has the potential to revolutionise the prevention and treatment of osteopenia in children. Working through increasing muscle mass and force, vibration training has the potential to both improve mobility and bone mass in children with a variety of disorders. The Institute has commenced trials of vibration training in children with Osteogenesis Imperfecta, Cystic Fibrosis and Mitochondrial Disorders. Protocols are also established for other disorders.

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32 Annual Report 2008-2009 | Stream 5 - Obesity, Metabolism and Nutrition

Snapshot: Obesity Research Group

“We are gathering evidence on what’s causing obesity, how do we treat those who are affected, and how do we prevent it in the first place.” - Professor Louise Baur

Childhood obesity: striving to tackle a chronic disease

Obesity is Australia’s fastest growing chronic disease in children and adolescents. A quarter of children are now overweight or obese which exposes them to a range of complications such as type 2 diabetes, obstructive sleep apnoea and the risk of heart disease.

Yet research into its causes, treatment and prevention is lacking, as is an effective government response to what is shaping up to be Australia’s most serious future health threat, says Professor Louise Baur, Head of the Obesity Research Group.

“There are a lot of negative attitudes in the community to obesity - we tend to blame the victim,” she says.

“The vast majority of clinicians are not trained in the recognition or management of this problem so it’s easy to ignore, a bit like mental health used to be. We are trying to raise the profile, to show what a significant problem it is.”

Professor Baur took a leading role in establishing both research and clinical work into childhood obesity in Australia. She runs Weight Management Services at The Children’s Hospital at Westmead, one of very few multidisciplinary obesity services offered to children in Australia.

Her research work includes studying gene expression of fat cells of children, epidemiological studies, systematic reviews of treatment interventions, clinical trials and population health intervention policy work.

Recently the Obesity Research Group contributed to a major Cochrane review of treatments, which found that lifestyle interventions led to improvements in weight, and that drug therapy offered additional benefit in adolescents with obesity.

A study into Loozit®, a community-based weight management program run by the Hospital, found it was successful in reducing waist circumference, improving patients’ lipid profile and raising their self esteem.

Courtney Renfrey hasn’t looked back since she started the program in April, 2008. The 16 year old lost 16 kg and is now encouraging her friends to get active and lose weight too.

“I weighed too much and my self confidence had gone right down,” she says. “You see yourself every day and don’t realise how heavy you’re getting. Now I’m a bit skinnier I’m not so self conscious.”

Professor Louise Baur, Director, Weight Management Services, The Children’s Hospital at Westmead

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33 Annual Report 2008-2009

Staff profile:Charmaine Tam, PhD student, Institute of Endocrinology and Diabetes

body, around the organs) is more ‘inflammatory’ than subcutaneous fat (the fat just under the surface of the skin). This means children with more visceral fat may be more likely to have metabolic and cardiovascular complications in later life.

She recently spent six months analysing her fat samples at a collaborator’s laboratory in Paris, where she found that fat tissue at a young age is a rigid structure, which becomes more loosely connected as children grow and even looser with excess weight gain. These changes may have profound effects on fat tissue biology.

After she finishes her PhD at the Kids Research Institute, Ms Tam is planning to travel to the United States to further her studies into obesity in humans.

Children’s fat cells help understanding of obesity

Overview The Children’s Hospital Institute of Sports Medicine’s (CHISM) research focuses on the assessment and efficacy of exercise in children with a range of chronic conditions. Collaborative research is undertaken with other departments at The Children’s Hospital at Westmead, including Rheumatology, Haematology and Endocrinology, as well as with other institutions in Australia.

One area of research involves patients who have musculoskeletal complaints, either acquired through injury or resulting from chronic conditions particular to children that affect their joints or bones. The other area involves children with serious illnesses who are prescribed exercise, for example to improve survival rates in cystic fibrosis or to increase fitness in children after long periods of hospitalisation.

Research AchievementsPrevious reports from Europe and the US reveal that children with haemophilia have lower levels of fitness and strength than their healthy peers. CHISM’s haemophilia research has focused on determining fitness and quality of life in Australian boys with

haemophilia, where prophylactic clotting factor is readily available. This research has already been completed and accepted for publication.

In addition to this, the research aims to quantify for the first time the increase in risk of bleeding associated with exercise in children with haemophilia so that children and their families can make informed decisions about sports participation.

CHISM has also studied muscle strength after steroid injections in children with juvenile idiopathic arthritis in the knee and elbow; fitness, motor skills and quality of life in children with juvenile idiopathic arthritis; the effect of two different dietary interventions on insulin resistance in adolescents; the effect of whole body vibration training on insulin sensitivity in overweight adolescents, and the effects of short-term high intensity exercise and plasma volume on cardiac performance in high school children.

In association with the Oncology Department’s Bone Marrow Transplant, CHISM is currently planning to investigate the benefits of exercise for patients undergoing bone marrow transplantation. This is the first investigation in this population.

Group Leaders: Dr Robert Parker, Department Head, Dr Carolyn Broderick, Staff Specialist Paediatric Sports Medicine, Dr Damien McKay, CHISM Honorary Research Fellow, Dr Gary Browne, Staff Specialist Paediatric

Sports Medicine, Nancy van Doorn, Paediatric Exercise Physiologist

Children’s Hospital Institute of Sports Medicine (CHISM)The role of exercise in treating sick kids

Fat tissue doesn’t just store energy and keep us warm. It’s an active organ releasing inflammatory proteins which can have adverse effects on the physiology of the whole body.

PhD student Charmaine Tam is conducting the world’s largest study of how children’s fat cells change with age and weight gain. Her work could one day help with the identification of the onset of obesity in children and the prevention of obesity-related complications.

Working at the Kids Research Institute has provided her with unique access to samples of abdominal fat, which are collected from children who are having elective surgery at The Children’s Hospital at Westmead.

Her early findings indicate that even in young children, visceral fat (the fat that is located deep within the

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34 Annual Report 2008-2009

Snapshot: Children’s Hospital Institute of Sports Medicine (CHISM)

“We are aiming to help parents make more informed decisions.” - Dr Carolyn Broderick

Is it safe for kids with haemophilia to play sport?

Every year David Taylor has to weigh up whether the benefits outweigh the risks of his son Jacob playing soccer.

Jacob loves the game, all his friends play and it keeps him fit. But as a child with haemophilia, a collision on the field could have serious health consequences.

Repeated bleeding into muscles and joints can ultimately destroy joints, resulting in pain and stiffness.

“As he gets bigger the risk increases,” David says. “I look at it on a year-to-year basis. Jacob thankfully has enough sense to not go in for the really bone crunching tackles and so far the benefits are outweighing the negatives.”

Anecdotally, children worldwide with haemophilia are less fit and tend to have a higher BMI than their healthy peers. It seems that their parents are wrapping them in cotton wool due to fear of injury.

However there has never been a study which quantifies the actual risks of sport and physical activity for children with haemophilia. The Children’s Hospital Institute of Sports Medicine, in collaboration with the George Institute and the Royal Children’s Hospitals in Melbourne and Brisbane, is the first centre in the world to launch such a study.

Participants will be contacted by text message once a week for a year to see if they have had a bleed in the preceding week and, if so, what activities they have been engaging in during the previous two days.

“At the moment families have to balance out the risks and benefits without any good scientific evidence,” says Staff Specialist in Paediatric Sports Medicine, Dr Carolyn Broderick.

“We know exercise is important for fitness and muscle strength but we don’t know what to tell them to do because no-one has ever assessed the risks. We are aiming to help parents make more informed decisions.”

In previous research, recently accepted for publication, Dr Broderick discovered that Australian children with haemophilia are as lean and fit as their peers in NSW. She hypothesised that this was due to the ready availability of prophylactic clotting factor which reduces incidence of bleeds as well as the encouragement the children receive from clinicians at The Children’s Hospital at Westmead to be physically active.

Dr Carolyn Broderick, Staff Specialist, Paediatric Sports Medicine

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35 Annual Report 2008-2009

Centre for Kidney ResearchImproving kidney health in children

Group Leaders: Professor Jonathan Craig, Head of Clinical ResearchAssociate Professor Stephen Alexander, Head of Laboratory Research

Overview The Centre for Kidney Research is working towards improving kidney health by providing insight into new treatments, methods of diagnosis, prevention and the enhancement of the quality of life of children with, or at risk of, kidney disease.

Its research aims to identify causes of disease, prevent and treat disease and evaluate the evidence for current treatments. It has an ongoing commitment to providing the latest evidence to enhance services, improve care and promote health. It also aims to teach others about kidney research and endeavours to make a significant contribution to local, national and international research communities, including its national network of kidney disease clinician-scientists.

Research AchievementsAll aspects of research are covered from basic to clinical and population health. This includes laboratory studies which look at genetic kidney diseases, treatments for a variety of kidney diseases and studies of rejection and tolerance of kidney, heart and islet transplants.

The Centre’s researchers have recently completed the world’s largest study investigating the effect of long term antibiotics on urinary tract infections in children, the PRIVENT trial. This multi-centred trial was conducted over 10 years, providing long-awaited evidence for practitioners that this treatment is effective.

New diagnostic methods for children with serious bacterial illness in the emergency room have been trialled in the FEVER Study, with outcomes currently being analysed.

The Centre is also involved in investigating bed-wetting and childhood daytime wetting. The NEAT Study is currently assessing the effectiveness of a

new wireless bed-wetting alarm device developed by their researchers.

Targeting risk factors that lead to kidney disease in the general population and high risk groups is also an important part of the Centre’s work. Aboriginal Australians have nine times the incidence of end-stage renal disease than non-Aboriginals. The Antecedents of Renal Disease in Aboriginal Children (ARDAC) Study is concerned with addressing this issue.

In addition, the Centre has been involved with the Cochrane Renal Group which is responsible for co-ordinating a register of all studies relating to kidney disease in adults and children. It has also produced the Caring for Australasians with Renal Impairment (CARI) Guidelines for nephrologists.

Targeting risk factors that lead to kidney disease in the general population and high risk groups is an important part of the Centre’s work

Research is undertaken into new treatments, new methods of diagnosis and improving the quality of life for children with kidney disease. The Centre for Kidney Research conducts laboratory work to help understand the genetic basis of kidney disease and the immune response to transplantation.

Stream 6 - Renal Medicine and Transplantation

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36 Annual Report 2008-2009

Snapshot: Centre for Kidney Research

“She effectively gave herself a bone marrow transplant.”

Demi’s miracle gives hope to transplant patients

Demi Lee Brennan is the first person in the world to take on the immune system and the blood type of her donor after she received a liver transplant. That means she will never have to take anti-rejection drugs again.

The theory is that some liver stem cells were transplanted with the donor organ. Usually the body’s own immune system rejects any cells that are transplanted but in Demi’s case the donor’s cells thrived and eventually replaced her own cells. She effectively gave herself a bone marrow transplant.

Her case received worldwide attention when it was published in the New England Journal of Medicine last year. Analysis of her T cells by microarray has been completed and further research is ongoing to determine exactly what happened in Demi’s case and eventually enable doctors to apply the process to transplant patients in a controlled way.

That could eventually remove patients’ lifelong reliance on anti-rejection drugs, even if they receive a completely mismatched organ, says Associate Professor Stephen Alexander, Head of the Centre for Kidney Research.

Both in nephrology and in the area of liver transplantation - where the Centre for Kidney Research has a research interest with gastroenterologist Dr Stormon and transplant surgeon Dr Albert Shun - these studies have profound implications for the long term health of children with transplants.

Demi received her donor liver when she was nine. She was healthy for six months but then fell ill as the new donor cells started to attack her own cells.

She was admitted to hospital for three months where her doctors realised her blood type had changed from O negative to O positive. Increasing her immunosuppressant medication did not improve her condition, so they took the risk of stopping the drugs altogether. The donor immune system took over and Demi’s health improved.

Demi has been off immunosuppression for more than five years, with no signs of rejection or other complications. She has finished school, works part-time and is planning to travel to the United States.

“I’m just so over the moon that eventually what happened to me could help so many lives,” she says.

Associate Professor Stephen Alexander, Head, Centre for Kidney Research (Laboratory)

Kate Geraghty/FAIRFAXPHOTOS.COM.

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37 Annual Report 2008-2009

This stream studies the immune system and ways of preventing infectious diseases. Research areas include immunisation and vaccine preventable diseases and this work informs policy regarding the spread of disease and epidemics. Other research looks at the transmission of perinatal infections from mother to unborn child; allergic disorders, which are increasing among Australian children, and the role of viruses in infectious disease.

Group Leader: Associate Professor Cheryl Jones, Department Head and Sub Dean, Research

Overview Perinatal infections acquired through transmission from the mother to the unborn child or newborn infant contribute to a large burden of childhood disease and disability and a large but unmeasured burden of miscarriages, stillbirths and premature births. However, these infections go virtually unrecognised in Australia.

The Centre for Perinatal Infection Research aims to reduce this disease burden by educating health care professionals and the community about diagnosis and treatment and to develop novel therapies to prevent or treat these infections.

One aspect of its research focus is to determine the reasons why the immune systems of newborn infants do not protect them against certain viral infections, like herpes simplex virus (HSV) or cytomegalovirus (CMV). It also performs internationally recognised national surveillance of perinatal infections to quantify the burden of disease they cause in Australian children.

Research AchievementsTo develop strategies to prevent infection and disease we need to first understand the body’s immune defences at the site of entry of the virus. To do this the Centre is performing studies of newborn immune cell responses to HSV. This project will lead to a better understanding of the earliest defences against viruses across all age groups and should facilitate the development of new therapies.

One recently described type of immune cells, regulatory T cells (T regs), play a vital role in controlling autoimmunity and excessive immune responses to infection. The Centre has recently derived from

transgenic mice important information about T reg modulation of antiviral responses which will lead to the development of therapeutics that provide life-long protective immunity without inducing autoimmunity.

The incidence, presentation and management of many perinatal infections in Australia are largely unknown. The Centre is running three collaborative studies of national surveillance through the Australian Paediatric Surveillance Unit (HSV, Hepatitis C virus and rubella) and collaborating on a fourth study of atypical mycobacterium. These studies are providing unique information on the burden of disease and epidemiology of infections and will be vital in the implementation of any preventative strategies such as vaccines. Collaboration with investigators overseas will compare the incidence of these infections in other countries (InOPSU).

There is currently no national routine antenatal screening program for infectious diseases in Australia. In particular, there is much debate as to the most appropriate antenatal screening policy for human immunodeficiency virus (HIV) and hepatitis C virus (HCV). It may also be appropriate to screen for other infectious diseases such as toxoplasmosis or past chickenpox exposure. The Centre is currently collaborating with the Macfarlane Burnet Institute to assess the current antenatal testing practice for infectious diseases, particularly HIV and HCV, used by general practitioners and obstetricians throughout Victoria and NSW.

Centre for Perinatal Infection Research Preventing and treating infections transmitted by mothers to their babies

Stream 7 - Infectious Disease, Immunology

These infections go virtually unrecognised in Australia

Kate Geraghty/FAIRFAXPHOTOS.COM.

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38 Annual Report 2008-2009

Overview The Department has two streams of research. The first relates to food allergy in children. The study of rare immunodeficiencies and T cell signalling is also conducted within the Department.

Allergic disorders, such as food allergies, asthma and atopic dermatitis, have increased dramatically over the past 20 years with over 30% of Australian children having some form of atopic disease. The Department’s research focus is on understanding how to prevent allergic diseases from developing and managing patients more effectively both at the individual and population based level.

Primary immunodeficiency disorders, whilst rare, require sophisticated investigative techniques for diagnosis and

intensive clinical management including immunoglobulin therapy and bone marrow transplantation.

Research AchievementsThe Department has published widely on food allergy and tolerance mechanisms over the past year.

It has conducted and published evidence of increasing food allergy presentations to the Emergency Department and increasing rates of food anaphylaxis. Another study supports the role for small amounts of egg incorporation in the diet of egg allergic children to subsequently outgrow egg allergy.

The study of Food Protein Induced Enterocolitis Syndrome (FPIES) - a rare non IgE food allergy disorder - has continued. Dr Alyson Kakakios continues her

Group Leader: Dr Alyson Kakakios, Department Head

Department of Allergy and ImmunologyInvestigating allergies and immune disorders in children

Group Leader: Professor Peter McIntyre, Department Head

Overview The National Centre for Immunisation Research and Surveillance of Vaccine Preventable Diseases (NCIRS) receives funding from the Federal Department of Health and Ageing to provide national reports on vaccine preventable diseases and vaccines, immunisation coverage and adverse events following immunisation. It also conducts national serosurveys and receives funding from NSW Health under a Memorandum of Understanding to provide specific additional support specific to immunisation programs in NSW.

In addition to this government funding, NCIRS has been successful in attracting funding for clinical and epidemiologic research from granting bodies including NHMRC and ARC and conducts clinical trials of specific vaccines under contracts with vaccine manufacturers, usually with other trial centres.

Research AchievementsWith respect to research and surveillance conducted under government funding agreements, a wide range of reports and epidemiologic studies were published during the previous year.

With respect to grant-funded and industry-funded research, influenza has been a particular focus in both children and adults. Ongoing studies have included an ARC-funded study on the economic and social benefits of treating influenza in aged care facilities and a study

under contract with CSL addressing the effectiveness of adult vaccination against influenza using the CSL vaccine.

Several new studies have commenced. These include examining viral resistance in influenza and hospitalisation secondary to influenza and another ARC-funded study into the effectiveness of influenza vaccination in children attending day care.

Most recently, NCIRS has commenced a H1N1 09 “swine” influenza vaccine immunogenicity trial with CSL and more than 400 children were recruited to a safety and tolerability study of seasonal influenza vaccine in 2009.

Two treatment trials of antivirals for influenza were also funded under NHMRC emergency funding provisions, one addressing whether resistance is less likely to develop if a higher dose of oseltamivir is used, while the other directly compares oseltamivir with zanamavir in relation to the risk of drug resistance developing on treatment.

Pertussis has been another focus of epidemiologic research and following a successful pilot study of acellular pertussis vaccine at birth, a NHMRC project grant was secured in 2008 to commence a larger multi-centre study in 2009. This study will compare monovalent pertussis vaccine given at birth with Hepatitis B vaccine at birth alone, with both groups of babies receiving a combination vaccine including pertussis at six weeks of age.

National Centre for Immunisation Research and Surveillance of Vaccine Preventable Diseases Preventing disease through vaccination

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39 Annual Report 2008-2009

Overview The Virology Research Group aims to increase understanding of the basic biology of viral infections in children - how viruses interact with cells and how they evade the body’s immune response.

There are two core areas of research: how the Flavivirus affects the regulation of immune cells and the role of viruses in childhood diarrhoeal illnesses.

Research AchievementsThe Virology Research Group has recently defined two pathways for up-regulation of immune recognition

molecules in mammalian cells. This work will inform understanding of immunity and assist with understanding vaccination risks.

The group is also collaborating with a mathematician to model the survival impact on the host of the alteration in the level of expression of immune recognition molecules.

Its work on the prevalence of rotavirus, norovirus and astrovirus among Australian children and a study of the genotypes that occur in our population will have implication for the future design of vaccines against these viruses.

Group Leader: Associate Professor Alison Kesson, Department Head

Department of Infectious Diseases and MicrobiologyUnderstanding viral infections

Staff profile:Associate Professor Cheryl Jones

Infections passed from mother to unborn child during pregnancy are a serious but under-recognised problem in Australia.

Every year, several children in every thousand suffer long term damage such as deafness, eye problems, cerebral palsy or mental retardation due to viruses that have been passed from their mothers, such as the herpes simplex virus (HSV) or cytomegalovirus (CMV).

However the impact of these viruses is virtually unnoticed by public health departments, health professionals and the community due to under-recognition of the nature and size of the problem, under-diagnosis, lack of awareness about prevention and poor uptake of guidelines and policies.

Associate Professor Cheryl Jones, a specialist infectious diseases paediatrician and head of the Centre for Perinatal Infection Research, has devoted her career to studying the mechanisms by which infections are transmitted to the foetus and newborn, with the aim or stopping this large burden of disability and disease they cause.

Her team has recently identified which type of herpes virus most commonly infects Australian newborn babies. It is also working on collaborations to understand why

babies’ immune systems cannot fight off the HSV virus and is looking at the incidence, presentation and management of perinatal infections in Australia.

“This work has important implications for how we would go about trying to prevent mums getting infected,” says Associate Professor Jones.

“It is also translated into policy, using our knowledge of the treatment of HSV and CMV with antiviral agents to produce guidelines nationally for the management of perinatal infections.”

Associate Professor Jones established a dedicated outpatient service at The Children’s Hospital at Westmead that aids families and health care providers with the diagnosis and management of mother-to-child infections.

Associate Professor Jones, also Sub Dean Research for the Discipline of Paediatrics and Child Health at the University of Sydney, is involved in the establishment of a Centre for Emerging Infectious Disease at the University. She is the recipient of the Sydney Medical School Award for Excellence in Postgraduate Research student supervision for her research and work supporting postgraduate research students.

Mother to child infections an under-recognised problem

involvement in the Early Prevention of Asthma in Atopic Children (EPAAC Study Group), which has published this year on worldwide variation in IgE sensitisation in children.

The Childhood Asthma Prevention study (CAPS) cohort continues to be examined. Parental compliance

with allergy primary prevention interventions has been studied and results published in 2008.

Dr Melanie Wong has continued the study of the STAT proteins T cell signaling pathways in health and disease.

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40 Annual Report 2008-2009 | Stream 7 - Infectious Disease, Immunology

Snapshot: National Centre for Immunisation Research and Surveillance of Vaccine Preventable Diseases

“The Children’s Hospital at Westmead has taken a leading role in research on the surveillance and treatment of human swine flu.”

Vaccine released after swine flu trialAs the swine flu H1N1 influenza pandemic swept the world, the Kids Research Institute played an important role in the development of a vaccine for children.

Forty-five participants were recruited by The Children’s Hospital at Westmead as part of a nationwide study to determine the efficacy and safety of the vaccine developed by CSL.

Its findings resulted in the release of a safe and effective vaccine for children in Australia and nearby countries. It may also be distributed in the Northern Hemisphere.

While the H1N1 virus results in a mild influenza in most people, a small percentage will become seriously ill or die. And because the great majority of people under 55 have no immunity to this new virus, it has infected two to three times more young people than seasonal influenza usually does, says Professor Robert Booy, Head of Clinical Research at the National Centre for Immunisation Research and Surveillance of Vaccine Preventable Diseases (NCIRS).

The trial was particularly welcome for participants such as the Shearer family, whose eldest daughter, six year old Ebony, was born very prematurely. Because she was

ventilated for more than 40 days, she has grown up with a chronic lung condition that could result in her becoming very sick were she to contract the H1N1 virus.

Her mother Natalie enrolled her other children, aged two and 10 months, in the trial so that the entire family would be immune and protect Ebony.

“We kept Ebony off school after the holidays, but I thought ‘I can’t keep her home forever’. I was quite worried before I found out about this trial,” says Natalie.

None of the children developed significant side effects to the vaccine, which was delivered in two doses, and they all remain healthy.

The Kids Research Institute has taken a leading role in research on the surveillance and treatment of human swine flu as well as its prevention, with three surveillance studies and two treatment studies in addition to the vaccine trial.

This research is a consequence of productive collaborations between NCIRS and the Australian Paediatric Surveillance Unit (APSU), both housed at The Children’s Hospital at Westmead, which provides the setting for high-quality teamwork.

Professor Robert Booy, Head, Clinical Research, National Centre for Immunisation Research and Surveillance of Vaccine Preventable Diseases (NCIRS)

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41 Annual Report 2008-2009

Overview The Australian Paediatric Surveillance Unit (APSU) contributes to the national surveillance effort and provides vital information on a range of rare infectious and vaccine preventable conditions, mental health, congenital and genetic conditions and injuries for which detailed national data are not available from other sources. It aims to translate research into policy which directly improves outcomes for children and adolescents.

A total of 1,330 paediatricians, or 92% of all paediatricians practising in Australia, and other child health clinicians participate in monthly APSU surveillance by providing de-identified data on children with rare diseases and conditions.

Research AchievementsAPSU’s surveillance system proved its effectiveness in responding at short notice to monitor epidemiological emergencies during the 2008 influenza season. The system is monitoring the swine influenza pandemic H1N1 in 2009.

In addition to ongoing surveillance for 14 rare diseases and conditions and the development of new surveillance studies, APSU is continuing to contribute to the development of innovative surveillance systems. These include the Paediatric Active Enhanced Disease Surveillance system (PAEDS), a hospital-based surveillance system piloted in four tertiary paediatric hospitals to pick up rare conditions and the Australian

Group Leaders: Professor Elizabeth Elliott, Director, Dr Yvonne Zurynski, Deputy Director

Australian Paediatric Surveillance Unit (APSU)A collaborative approach to the study of rare childhood diseases

Research into the delivery of health services aims to provide an evidence-based approach to the way we treat sick children. It incorporates national surveillance into childhood illness and injury; improvements in nursing services, and supports education for students and staff at the University of Sydney’s Faculty of Medicine. Clinical sciences research spans care for children injured in traumatic circumstances; respiratory medicine; sleeping disorders and SIDS; nuclear medicine; care for sick newborn babies, and rehabilitation for children with brain injuries and cerebral palsy.

Stream 8 - Clinical Sciences and Health Services Delivery

Staff profile:Dr Nicholas Wood, Clinical Fellow, National Centre for Immunisation Research and Surveillance of Vaccine Preventable Diseases (NCIRS)

Newborn babies are particularly vulnerable to pertussis (whooping cough) but they currently don’t develop adequate immunity until they are about five months old as they are not inoculated until two and four months. That’s because initial studies indicated that giving the injection too young might result in patients ultimately becoming immune to the vaccine.

Now that vaccines have changed and ways of assessing immunity have improved, the recent pertussis epidemic in NSW has highlighted a need to protect babies as soon as possible. Staff specialist Dr Nicholas Wood has recently received a NHMRC grant to investigate the possibility of vaccinating babies at birth.

Centres in Sydney, Melbourne, Perth and Adelaide will also look at “cocoon vaccination” – where babies

are protected by vaccinating the people with whom they come into contact such as their parents and health care workers.

“Ultimately we could recommend that the schedule for pertissus vaccination be changed nationally to protect thousands of newborn babies from what is for them a potentially fatal infection,” says Dr Wood.

Dr Wood, who has spent time working as a paediatrician in Africa and China, has a special interest in maternal and neonatal vaccination. He is involved in the Immunisation Adverse Events Clinic and Refugee Clinic at The Children’s Hospital at Westmead.

His other current research interests include the longevity of hepatitis B vaccination in infancy.

Studying how to protect young babies from whooping cough

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42 Annual Report 2008-2009

Overview Kids Heart Research is a team of scientists and doctors working together to address clinical problems in the area of cardiac health. Congenital heart diseases affect one in every 100 children, with problems ranging from ‘holes in the heart’ to more complex conditions.

Its research efforts are focused on two main areas: improving understanding of the genetic basis of congenital heart disease and finding ways to make heart surgery safer and more effective.

Research AchievementsWater accumulation in the body’s tissues, including the heart, was traditionally thought to contribute to poor recovery after surgery. Using a range of experimental models, Kids Heart Research has demonstrated that the link between swelling and impaired heart function is not as important as previously thought. The work highlighted the detrimental effect of depriving the heart of its usual blood supply (ischaemia).

It is likely that the way infant cardiac surgery is conducted will be altered as a result of these important findings. Ongoing research is focused on reducing the impact of ischaemia, which is a

necessary part of open heart surgery, to speed recovery and ultimately make surgery safer. The most exciting development in this area is a therapy new to paediatric cardiac surgery that is currently being tested in a pre-clinical trial.

In addition to many local collaborative projects, Kids Heart Research has contributed hundreds of samples from the Kids Heart Research DNA bank to two multi-centre international genetics studies. Both investigations will perform genome-wide association analyses that aim to discover new genes associated with the development of congenital heart disease.

Other work centres on how having a heart problem can affect children in the long term. Kids Heart Research is working closely with the Grace Centre for Newborn Care to track the development of children who underwent heart surgery in the first 90 days of life. This study involves detailed follow-up of children to preschool age, including intricate brain imaging.

It is likely that the way infant cardiac surgery is conducted will be altered as a result of these important findings

Group Leader: Associate Professor David Winlaw, Department Head

Kids Heart ResearchBeating heart disease in kids

Maternal Outcomes Surveillance System (AMOSS) which aims to provide detailed, systematically collected data on serious but rare outcomes related to birth and pregnancy.

In 2008 APSU convened a National Task Force for Rare Diseases. Its new program of research on the impact of rare diseases on families, clinicians and health services will inform the development of co-ordinated plans to address rare diseases and information resources for families and clinicians.

APSU continues to inform public health policy and clinical practice. For example, it is monitoring the incidence of neonatal, congenital and severe complications of varicella after the introduction of the varicella vaccine; data collected on Fetal Alcohol Syndrome informed NHMRC Australian Alcohol

Guidelines and data collected on seatbelt-related injuries informed the 7th Amendment to the Australian Road Rules.

APSU has addressed surveillance gaps among indigenous children and refugees by mounting surveillance for conditions particularly relevant to these groups, such as acute rheumatic fever and Vitamin D deficiency rickets.

APSU was the first unit of its kind in the world to undergo systematic evaluation and meet key criteria set by the Centers for Disease Control and Prevention.

APSU continues to inform public health policy and clinical practice

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43 Annual Report 2008-2009

Overview The Centre for Trauma Care, Prevention, Education and Research (CTCPER) is the core centre for trauma services at The Children’s Hospital at Westmead. Its mission is to provide high quality services to children and adolescents injured due to traumatic events, to play a leading role in the prevention of injury among children and adolescents and to conduct groundbreaking research in trauma care, prevention and education.

The Centre also plays an important role in training health professionals in trauma-related clinical skills and providing safety information and education to the public.

Research AchievementsCTCPER is a member of a large research consortium

investigating the causes of car crash-related injuries among young drivers in NSW. A large-scale study in which more than 20,000 young drivers were followed for three years after obtaining their provisional licences has just been completed and the research team is currently conducting data analyses.

Earlier research into the factors that put young people at risk of motor vehicle accident injuries identified the number of passengers carried by drivers under the age of 25 as a significant risk factor. These results, together with those of subsequent research, led to road and traffic authorities in New Zealand and Australia implementing legislation to restrict the number of passengers carried by these drivers. Recent road statistics indicate a decline in motor vehicle-related crashes and injuries.

Results obtained from CTCPER’s school bus-related injuries study were instrumental in the implementation of 40 km/h zones around school areas during the time of day when most school children arrive at and leave school.

There is an on-going research program into ways to improve the care and management of traumatically injured children admitted to hospital. A study on the transfer of paediatric trauma patients provided evidence for the Statewide Trauma Management Committee as a base for changing the statewide trauma transfer policy.

The Centre has also developed a state-wide network to investigate preventive factors for children falling out of residential buildings. A state-wide intergovernmental working task force has been established and has also been officially launched by the NSW Health Minister to tackle this specific issue of childhood injury.

Concurrently, the Centre has developed a collaborative group to examine ways of preventing off-road motor cycling injuries among children and adolescents. A symposium has been held to discuss potential research directions for the prevention of this particular problem.

Group Leaders: Professor Danny Cass, Director, Dr Lawrence Lam, Scientific Director

Centre for Trauma Care, Prevention, Education and ResearchLeading the way in trauma care and research

The Centre plays an important role in training health professionals in trauma-related clinical skills

Overview Research on lung disease within the Department of Respiratory Medicine is carried out under the auspices of the Respiratory Medicine Research Group (RMRG), which was established in 1998 as The Children’s Chest Research Centre. Its well-functioning clinical research team conducts research projects which have proved to have significant clinical applicability. It has also established a number of important internal and external research collaborations.

Research focuses on the causes, diagnosis, investigation and treatment of respiratory illness in children, in particular cystic fibrosis, asthma and the respiratory consequences of extremely preterm birth.

Research AchievementsIn addition to making a significant contribution to the foundation of the Exercise Testing Laboratory located within CHISM, members of the RMRG have been involved in the acquisition of equipment in the Kids Activity Research Centre (KARC). This has

Group Leaders: Professor Peter van Asperen, Department Head, Dr Karen McKay, Overseeing Clinical Trials,Dr Hiran Selvadurai, Overseeing Exercise and Physiology Studies

Respiratory Medicine Research GroupHelping kids breathe easier

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44 Annual Report 2008-2009

Overview The SIDS and Sleep Apnoea Research Group is associated with the Sleep Disorders Unit at The Children’s Hospital at Westmead and the David Read Laboratory at the University of Sydney. The current clinical sleep unit was established as a research facility with the assistance of the (then) National SIDS Council of Australia.

The Group’s laboratory research focuses on risk factors for SIDS - nicotine exposure and intermittent hypoxia. Its clinical research focuses on the causes, complications, investigation and treatment of sleep apnoea and sleep disorders in children. At The Children’s Hospital at Westmead, the Group is investigating many aspects of obstructive sleep apnoea (OSA) in infants and children using sleep studies to document the presence and severity of OSA. It is currently placing considerable focus on determining the effectiveness of treatment interventions, which includes the use of medication, surgery or nasal mask CPAP.

Research AchievementsWith the support of the Australasian Sleep Trials Network, the Group has developed a multicentre Paediatric Sleep Centre collaboration across Australia. It has established the protocol to undertake a definitive study of whether adenotonsillectomy improves the IQ in children with OSA and is currently seeking funding for this project. It has been a major advance for the SIDS and Sleep Apnoea Group to be nominated as the co-ordinating centre for a multicentre Paediatric Research

Group in Australia and marks the commencement of rigorous scientific evaluation of treatment in Paediatric Sleep Medicine through randomised trials.

A main focus of laboratory-based research at the University of Sydney is the significance of intermittent hypoxia and exposure to cigarette smoke as risk factors for SIDS. The Group has helped develop new methods to study the brain tissue of piglets and infants who died from SIDS. Other work is looking at how preeclampsia or prematurity can affect the development of the brain.

PhD projects include providing information about how the presence of a cleft lip and/or cleft palate increases the risk for sleep-disordered breathing in infants, how this affects the development of their ventilatory control and how this impacts on their neurocognitive development.

In the area of obstructive sleep apnoea (OSA), the Group is studying the effectiveness of adenoidectomy, initially by questionnaire, followed by an assessment of neurocognitive outcomes with the use of sleep studies.

Where adenotonsillectomy is not adequate to treat OSA, nasal mask CPAP is used to treat the condition in children. The Group is studying how the presence of obstructive sleep apnoea interacts with obesity to influence cardiovascular function in children.

It is placing considerable focus on determining the effectiveness of treatment interventions

Group Leader: Associate Professor Karen Waters, Department Head

SIDS and Sleep Apnoea Research GroupFirst sleep!

established exercise testing as an important tool in the assessment of children with chronic lung diseases and other chronic diseases which may impact on cardiopulmonary functioning.

As a result of a long term follow-up of children born extremely prematurely, there is some evidence that only a mild impairment of lung function can be expected in at least a proportion of these children by the age of 10. This evidence can now be added to the body of information provided to the parents of extremely premature neonates when they are making crucial decisions about their infant’s care.

RMRG contributed to a study of over 1,000 infants showing that the asthma medication Montelukast is of no benefit in treating bronchiolitis despite the inflammation in the airways in these diseases having similar mechanisms. It has also been involved with

the evaluation of formulations of antibiotics developed specifically for nebulisation for children with cystic fibrosis, at least one of which is the subject of a marketing application in Australia. It has also been involved in studies with dry powder mannitol, both as a diagnostic test for children with asthma and as a treatment for children with cystic fibrosis.

An up-to-date data registry of cystic fibrosis patients was introduced in early 2008. Its data enables doctors to monitor the health of children after changes are made to their care, for example, the effect of a dietician on managing the children’s nutrition. The registry also enables the production of an up-to-date clinical report for each patient prior to clinic visits. Such reports have been shown to engage families more in goal setting for their children.

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45 Annual Report 2008-2009 | Stream 8 - Clinical Sciences and Health Services Delivery

Snapshot: SIDS and Sleep Apnoea Research Group

“It’s made him a totally different child.” - Sarah Meudell

A good night’s sleep for a healthy heart Jack Meudell has obstructive sleep apnoea - an increasingly common condition in children which means he stops breathing for several seconds repeatedly during the night.

Now eight, Jack has slept in his mother’s bed every night since he was a toddler. He never falls into a deep sleep and his behaviour and attention span have been affected.

According to research being conducted by the SIDS and Sleep Apnoea Research Group it could also be affecting his heart.

Sleep apnoea in adults is associated with hypertension and stroke because of the increased effort required by the heart due to repeated oxygen deprivation. But there have been no studies into how it affects children’s ability to respond to exercise when they are awake.

PhD candidate Carla Evans is studying cardiac function in children with sleep apnoea by measuring their heart rate and the blood’s ability to circulate blood through the body before and after treatment.

Initial assessments showed that children with sleep apnoea cannot show as much increase in their cardiac activity during exercise as those without sleep apnoea.

By following those children after treatment of their apnoea, the team is finding out how quickly their exercise function returns to normal if their apnoea is treated. If sleep apnoea in children remains untreated the damage to their heart and lungs would probably continue to deteriorate.

Most sleep apnoea in children is caused by enlarged adenoids and tonsils and treatment with a CPAP machine is used until surgery can be undertaken to correct these problems. For Jack, receiving a CPAP machine earlier this year resulted in a dramatic improvement in his day-to-day life.

“It’s made him a totally different child,” says his mother, Sarah.

“He sleeps better. He wakes up in the morning alert and happy, and his behaviour at school has improved out of sight.”

The Children’s Hospital at Westmead, which runs one of Australia’s largest and busiest sleep units, has also evaluated current treatments of sleep apnoea and is looking at whether treatment of sleep apnoea improves the learning and behaviour problems often seen in these children.

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46 Annual Report 2008-2009

Overview The Department of Nuclear Medicine is the leading clinical and research paediatric nuclear medicine centre in Australia.

Its research is focused on three areas: the clinical evaluation of nuclear medicine and bone mineral density techniques relating to improvements in patient management; basic sciences (radiopharmacy) such as the development of new radiopharmaceuticals and applications; and basic sciences (physics), the development and/or modification of imaging equipment and techniques for new applications or improvements in current uses.

Research AchievementsThis year the Department conducted a clinical review of PET/CT studies in patients with paediatric lymphoma and primary bone tumours. It found that this imaging showed more lesions and was more specific than conventional imaging, which has enabled

clinicians to better gauge the response to treatment in their patients and adjust their patient management accordingly.

The Department undertook the world’s largest review of paediatric lymphoscintigraphy, reviewing 126 cases and documenting scan patterns. It will enable clinicians to predict lymphodaema in non-symptomatic limbs and lead to more specific treatment options.

In the area of basic sciences, the Department has evaluated the use of a new agent for PET scans in inflammatory arthritis, neuroblastoma and infection.

It also received a NHMRC Development grant for Westmead Hospital and The Children’s Hospital at Westmead to develop a motion correction system for PET/CT in paediatric patients.

There are several ongoing projects relating to assessment of bone mineral density and body composition. This research is a collaboration between the Department and clinical units such as endocrinology, genetics and orthopaedics.

Group Leader: Professor Robert Howman-Giles, Department Head

Department of Nuclear MedicineLeading the way in the use of nuclear medicine for children

Overview The Grace Centre for Newborn Care is a neonatal intensive care unit which cares for approximately 600 infants each year from NSW, the ACT and surrounding Pacific islands. The unit specifically cares for babies with cardiac and surgical problems or complex medical conditions.

As part of the Centre’s mission to improve care and outcomes for babies and their families, it encourages novice researchers and postgraduate research degree enrolment. The Centre’s research focuses on clinical issues, particularly on how to improve the quality of care and long term outcomes of newborn babies both in the Neonatal Intensive Care Unit (NICU) and after they go home.

Research AchievementsAmong several competitive research grants achieved this year is funding from the March of Dimes Birth Defects Foundation in the USA to study long term quality of life in very small babies who have survived complex surgery at ever younger ages. The DAISY (Development after Infant Surgery) Study addresses the effects of infant surgery, aiming to identify the range of long term outcomes and enabling early interventions.

The Centre is also examining the social and emotional burden on families of having an infant who requires neonatal surgery. This includes a study of post traumatic stress in parents who have a baby in intensive care and following discharge.

Often babies in the NICU are exposed to many potentially painful procedures as well as pain as a result of neonatal surgery. The Centre is part of a national project to close the evidence/practice gap for pain management practices in the post-operative period in newborns. As part of this, a study was undertaken to obtain information on the practices and beliefs of junior doctors regarding neonatal pain and to compare this to best evidence.

Several other research projects are underway. The Centre is examining the effects of up-to-date nursing knowledge and decision-making skills on patient outcomes, a collaborative project with all the NICUs in NSW. It is also reviewing the anti-inflammatory protein, Clara Cell Secretory Protein, the deficiency of which in preterm babies contributes to premature lung disease and assessing the role of diagnostic ultrasound prior to transport from peripheral hospitals on making sure the babies reach the unit in the best possible condition.

Group Leaders: Associate Professor Nadia Badawi, Co-Head, Grace Centre for Newborn CareAssociate Professor Kaye Spence, Clinical Nurse Consultant, Neonatology

Neonatology and the Grace Centre for Newborn Care Research UnitImproving the care for newborn babies and their families

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47 Annual Report 2008-2009

Snapshot: Neonatology and the Grace Centre for Newborn Care Unit

“Our work has highlighted a desperate need for ongoing care and follow up of babies who have surgery.” – Associate Professor Nadia Badawi

What’s next for this tiny survivor?

Yanni Bulter was born with a congenital left sideddiaphragmatic hernia, a condition which meant hisdiaphragm failed to develop properly in the womb andhis lungs could not work properly. A decade ago, hewould have had a 50-50 chance of surviving.

But advances in neonatal treatment mean that morethan 90% of infants now survive the condition. After Yanniwas transferred to The Children’s Hospital at Westmead,which treats NSW’s sickest babies, his hernia was surgicallycorrected and he quickly began to recover.

Newborn survival has increased dramatically in recent years. Fifteen years ago 17% of babies who were born with congenital defects died. Now more than 97% survive, even though sicker and smaller babies are routinely treated.

But no-one has gauged the long term effects of having surgery so young. Associate Professor Nadia Badawi, a consultant neonatologist at the Kids Research Institute, is leading research on the long term quality of life of these tiny patients.

The Daisy Study, funded by the US March of Dimes Foundation, is following up every baby in NSW who has surgery. It is comparing outcomes of healthy babies with those who have heart surgery and those who have other types of surgery.

While most are doing well at one year, it has found that surgery in infancy - especially heart surgery - can predispose a small number of babies to problems such as developmental delay or cerebral palsy. This may be due to the anaesthetic, the trauma of surgery, or because babies with one birth defect are more likely to be small or to have another defect.

Associate Professor Badawi is hoping to open a follow up clinic for these babies to provide them with the early intervention and long term care they need.

“The first question parents ask is ‘Is my baby going to make it?’ and the next is ‘What’s its quality of life going to be?’ The problem has been, we don’t know,” she says.

“Our research aims to tease out exactly what it is about these kids that predisposes them to later problems and then ultimately to prevent them.”

Associate Professor Nadia Badawi, Co-Head, Grace Centre for Newborn Care

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48 Annual Report 2008-2009

Overview The Discipline of Paediatrics and Child Health is a leader in medical education and medical education research.

Located at The Children’s Hospital at Westmead, the Discipline is a department of the Sydney Medical School at The University of Sydney. Its large team of teachers, tutors and examiners - comprising medical, nursing and allied health staff both within and external to the Hospital - plays a significant role in providing a high quality education and training environment to the more than 350 medical students who undertake their paediatric rotation at The Children’s Hospital at Westmead each year.

Activities include investigating evidence-based methods of information delivery to students and medical professionals; participating in curriculum development for medical students; co-ordinating placements for elective students and junior medical staff; the local management and co-ordination of postgraduate students and their supervisors, and the online delivery of a Masters in Medicine (Paediatrics) course.

The Discipline offers clinical and research staff the opportunity to apply for conjoint and clinical titles and provides valuable teaching and professional development opportunities. Members of the Discipline are in the unique position of being able to address questions raised in the clinical setting through high quality research. The Discipline has also created a vibrant and productive environment capable of attracting new research groups.

There has been an increase in the number of clinicians participating in research, teaching and education and the number of clinical and conjoint appointments within the Discipline has almost tripled over the past few years. There are now 250 Hospital staff with a formal affiliation with the Discipline from a large range of clinical and research departments.

In the past year academic members of the Discipline were investigators on successful grants worth over $3.15 million and published 188 articles in high quality paediatric journals.

Group Leader: Professor Kathryn North, Department Head

The Children’s Hospital at Westmead Clinical School: Discipline of Paediatrics and Child Health, Sydney Medical School, University of SydneyManaging and preventing obesity in Australia’s youth

Overview The Nursing Research and Practice Development Unit aims to ensure that care delivered at The Children’s Hospital Westmead meets the needs of patients and their families.

The Unit is currently undertaking a five-year practice development program which is progressively being introduced to clinical units throughout the Hospital. Activities in the program encourage staff to reflect on current practice, identify the need for change and challenge themselves and each other to do better, where possible.

It is envisaged that engagement in these types of activities will lead to improved teamwork and staff satisfaction, sustainable changes in practice and ultimately improved family-centred care.

Research AchievementsThe Unit is leading an international project which is reviewing the theory behind how practice development is evaluated.

It is working in partnership with state and international organisations on a research project

involving the practical application of practice development principles (REACH). REACH offers a pathway to lifelong learning through an effective appraisal process, an attributes framework, a personal development contract, reflective practice, the development of a portfolio and formal and work-based learning opportunities.

The Unit is also examining the factors that increase facilitator development. It is leading and evaluating training and opportunities for development, for example, via week-long schools each year that enable health professionals to learn about practice development methodology and methods and develop skills as facilitators.

A framework of Teamwork-Learning-Change, developed by Professor Val Wilson, is being used to lead the State-wide implementation and evaluation of the Essentials of Care Program (NSW Health initiative) in paediatrics. This program of work involves all members of the team in realising an espoused philosophy of family-centred care.

Group Leader: Professor Val Wilson, Department Head

Nursing Research and Practice Development Unit Achieving person-centred care

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49 Annual Report 2008-2009 | Stream 8 - Clinical Sciences and Health Services Delivery

Group Leaders: Professor Elizabeth Elliott, DirectorDr Diana Thomas, Scientific Director

Centre for Evidence-Based Paediatrics Gastroenterology and Nutrition (CEBPGAN)

Translating scientific research into evidence-based practice

glycaemic load (GL) diet compared with those on a high GI or GL diet or other diet. This work became one of the four most accessed reviews for the year in the Cochrane Library and its data has been incorporated into the European Clinical Practice guidelines for the management of obesity in adults. At least one large randomised controlled trial has since been designed based on the follow-up recommendations from the review, to elicit the longer term impact of low GI diets on obesity.

Another Cochrane review showed there was a significant improvement in blood glucose control with the low GI or low GL diets compared with a high GI or GL diet or other diet in people with diabetes. Numerous summaries and articles based on the review have been published.

Other reviews have studied the important role of exercise in improving blood glucose control in people with type 2 diabetes mellitus; interventions for haemolytic uraemic syndrome and thrombotic thrombocytopenic purpura and non-surgical interventions for eosinophilic esophagitis.

Overview The Centre’s focus is on the translation of scientific research into medical practice. CEBPGAN informs evidence-based practice by critically appraising the literature and conducts systematic reviews on treatments relevant to paediatric gastroenterology and nutrition.

CEBPGAN runs teaching seminars on evidence-based practice for clinicians and provides an advisory service for clinical researchers. It has developed databases of randomised controlled trials, systematic reviews and clinical practice guidelines relating specifically to paediatric gastroenterology and nutrition. These provide assistance to clinicians, such as paediatricians and dietitians, a resource for researchers and assistance for the media and the public worldwide.

Systematic reviews of the literature registered with the relevant review group in The Cochrane Collaboration are undertaken on subjects relevant to The Centre’s focus.

Research AchievementsA Cochrane review showed there was more weight loss in obese people on a low glycaemic index (GI) or

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50 Annual Report 2008-2009

Overview The Department of Rehabilitation is a busy clinical unit which currently serves over 3,000 patients. Treatment and research are conducted in the areas of cerebral palsy, brain injury, spina bifida and limb deficiency.

With no dedicated research staff, over 60% of the clinical staff are nevertheless actively involved in clinical research. The Department’s 30 current projects include a state-wide evaluation of community outcomes for children with brain injury; pharmacological and cognitive treatment options for children following acquired brain injury; assessment of quality of life in children with cerebral palsy and service delivery for children and families living with disabilities.

Research AchievementsAs one of the largest centres in Australia providing botulinum toxin therapy for children, the Department’s

research in this area has contributed greatly to international knowledge of the outcomes and safety profile of this important treatment modality for children with cerebral palsy.

The Department is in the early stages of recruiting for a study of deep brain stimulation in children with cerebral palsy. This is an exciting opportunity to be at the forefront of work in a new innovation in the management of the challenging problem of dystonia. A national database to monitor outcomes and adverse events following intrathecal baclofen treatment for spasticity and dystonia is being planned.

Other research concerns the evaluation of interventions such as serial casting, exercise, constraint induced therapy or orthotics and the application of a play therapy-based intervention for the management of anxiety in children undergoing botulinum toxin injections.

Group Leaders: Dr Stephen O’Flaherty, Department HeadDr Angie Morrow, Research Committee Chairperson

Rehabilitation Research Group A better quality of life for children with disabilities

Staff profile:Dr Laurence Cantrill, Advanced Microscopy and Imaging Specialist

The large research community at the Kids Research Institute could not operate without a body of dedicated support staff. Microscopist Dr Laurence Cantrill is responsible for the Microscopy Facility that enables scientists to study how the tiniest objects in the body work.

The Facility contains a suite of instruments which provide fluorescence and confocal microscopy, live cell imaging and laser capture microdissection microscopy.

Dr Cantrill maintains the confocal microscope, which can look at objects thousands of times smaller than 1 mm. Other instruments available at the Westmead Research Hub, such as the electron microscopes, are used to view even smaller objects.

Trained as a plant biologist, Dr Cantrill was attracted to the Kids Research Institute by the chance to run and improve a top quality microscopy facility as well as collaborate on a large variety of projects.

He is planning to expand the Facility by replacing the existing confocal microscope and introducing correlative microscopy in collaboration with colleagues across the Westmead campus. This technique brings together light microscope and electron microscope technology, enabling researchers to observe living cells then very quickly freeze them and look at particular areas in the same cells in much higher detail.

“It will show them things they have never seen before - they will be able to see disease processes right down at the molecular level,” Dr Cantrill says.

“This facility is unique in a hospital setting but I’d like to advance it further by bringing new technology in. Its appeal for me is that I get to work on a wide range of different projects and have access to cutting-edge technology and the beautiful images of life that it creates.”

Laurence’s beautiful images of life

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51 Annual Report 2008-2009

Snapshot: Rehabilitation Research

“The cure for cerebral palsy isn’t around the corner tomorrow - but what we can do is dramatically improve quality of life and children’s ability to participate in life.” - Dr Angie Morrow

Rachel takes on the world

Cerebral palsy affects one in 400 babies born in Australia. A non-progressive disorder of the brain, it mainly affects how children move - their muscles may be stiff or tight, they may shake, or they may have seizures and other impairments.

A common problem for many of these children is dystonia: abnormal tone which causes painful muscle contractions and results in children being floppy or stiff and twisted.

An exciting new innovation in treating dystonia is deep brain stimulation. The Department of Rehabilitation is in the early stages of recruiting for a randomised controlled trial of this therapy which will provide a major contribution to world understanding in this area.

Hundreds of children at the The Chidren’s Hospital at Westmead have now been successfully treated with botulinum toxin (Botox) therapy, which is used to relax muscles in the legs every four to six months and enable children to walk properly. The Kids Research Institute is currently auditing long term outcomes of these patients to establish botulinum toxin’s safety.

Another treatment which has changed many children’s lives is the use of an Intrathecal Baclofen Pump, a device implanted in the abdomen which releases computer-controlled doses of the drug baclofen directly into the spinal fluid to loosen the muscles.

For Rachel Sutton, the treatment has worked spectacularly. Since she was fitted with a pump six years ago, the now 16 year old no longer has painful muscle spasms that break her equipment and it’s much easier for her to enjoy teenage activities such as shopping and swimming.

She can now use her eyes and head to communicate and was able to pass her School Certificate last year at a mainstream high school.

“It’s been magnificent,” says her mother, Margaret. “Before her muscle tone was so stiff it was impossible for her carers to care for her.

“Doctors at The Children’s Hospital at Westmead listen to you - every one of them really wants to learn.”

Dr Angie Morrow, Staff Specialist, Department of Rehabilitation

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Staff and students

Staff and students

Research Office Staff

Director A/Prof Chris Cowell (Acting)

Research and Development Manager Anne O’Neill (to January 2009)

Fundraising and Revenue Manager Trish van Leeuwen (to May 2009)

Research Governance Manager Carolyn Casey (from July 2009) James Cokayne (from February 2009)

Ethics Manager Carolyn Casey (to July 2008) Eleanor Thackray (from August 2008)

Clinical Trials Pharmacist Pathma Moodley

Clinical Trials Co-OrdinatorDr Kimberly Lilischkis

Grants and Scholarships OfficerAmelia Hill (to July 2008)Lyndsey Bray (from September 2008)

Executive Support OfficerAmelia Hill (from July 2008)

Ethics Administration AssistantJessica Moller

Facility Support OfficerLyndsey Bray (to September 2008)Anne Sutherland (from December 2008)

StatisticianDr Cornelis Biesheuvel (to August 2008)Dr Frederica Barzi (from June 2009)

IT Support OfficerImran Hussain

Engineering ManagerStephen Gorrey

Laboratory ManagerMatthew Laver

Laboratory OfficerTrish McGregor

Laboratory AssistantDeidre Shiel

Advanced Microscopist & Imaging SpecialistDr Laurence Cantrill

Electron MicroscopistDr Marko Nykanen

Transgenic Facility ManagerRebecca Reilly

Senior Technical Officer Karen Knight

Animal TechniciansHeidi LeschmannKevin KerrVeronica Pollero

Adolescent Medicine and Eating Disorders

StaffDr Sloane Madden - Senior Staff SpecialistA/Prof Michael Kohn - Senior Staff SpecialistA/Prof Simon Clarke - Senior Staff SpecialistDr Paul Rhodes - Senior Lecturer, The University of SydneyAndrew Wallis - Senior Social Worker/PhD Student

StudentsRani Ellison - DCP StudentAinslie Hatch - PHD Student

Adolescent Medicine Psychologists

StaffHelen Bibby - Clinical Psychologist/Researcher Jane Miskovic - Psychologist/Eating Disorder Research Co-ordinator Popi Zappia - Individual and Family Therapist/ Psychologist TeenLinkNatasha Comarin - Psychologist Fleur Harrison - Psychosocial Research Assistant

StudentsRani Ellison - Doctoral PsychologyAinslie Hatch - Doctoral PsychologyAlex Withers - Doctoral Psychology

Adolescent Medicine Ronalda Hoffman - Senior Social Worker HeadAndrew Wallis - Senior Social Worker

Allied Health

AudiologyRosemary Douglas - Head Audiologist

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Staff and students

Staff and students

Kids Health

Candace Douglass - Department Head Dietetics

StaffSusie Burell - Obesity Service DietitianKerryn Chisholm - Obesity Service DietitianChristie Graham - CF Dietitian Rebecca Fisher - Oncology BMT DietitianGabriella Heruc - Oncology DietitianKatie Barwick - Renal Dietitian Barbara Dennison - Allergy DietitianSusan Thompson - Senior Dietitian, Genetic Metabolic Disorders Service

Occupational Therapy

StaffMargaret Wallen - Senior Occupational TherapistProf Anita Bundy - Honorary Appointment Paula Bray - Occupational TherapistLisa Bidgood - Occupational Therapist

Pharmacy

StaffPenny Thornton - Pharmacy Services ManagerPeter Barclay - Deputy Director of PharmacyPathma Moodley - Clinical Trials and Drug information Senior PharmacistJoyce Liew - Clinical Pharmacist

Physiotherapy

StaffAlison Chivers - Physiotherapist, Talipes Jennifer Follett - Head of Department Kerry West - Deputy Head Verity Pacey - Senior PhysiotherapistKelly Evans - Senior Physiotherapist, OrthopaedicsBronwyn Thomas - Senior Physiotherapist, RehabilitationJan Hancock - Senior Physiotherapist, RehabilitationAlison Elliott - Physiotherapist, Cystic FibrosisJenny Wu - Senior Physiotherapist, Cerebral PalsyKaren Bau - Senior Physiotherapist, Cerebral Palsy

StudentsKelly Evans - Masters StudentAnna Middleton - PhD StudentVerity Pacey - PhD Student

Social WorkSue Foley - Senior Social WorkerJenny Rose - Senior Social WorkerRobyn Lamb - Senior Social Worker and Co-Head of CPU

Martine Simons - Senior Social WorkerSuzanne Benson - Senior Clinical Psychologist and Clinical NeuropsychologistMelissa Loos - Social Worker Surita Stipp - Social WorkerAngie De Casanove - Social Worker

Anaesthetics

StaffDr David Baines - Department HeadDr Neil Street - Deputy Head Dr Stephanie Aplin - Consultant AnaesthetistDr Jenny Chien - Consultant AnaesthetistDr Michael Cooper - Consultant AnaesthetistDr Jonathan De Lima - Consultant AnaesthetistDr Peter Gibson - Consultant AnaesthetistDr Sue Hale - Consultant AnaesthetistDr Donald Innes - Consultant AnaesthetistDr Ramanie Jayaweera - Consultant AnaesthetistDr Sarah Johnston - Consultant AnaesthetistDr David Kinchington - Consultant AnaesthetistDr Mark Lovell - Consultant AnaesthetistDr Ian Miles - Consultant AnaesthetistDr David Murrell - Consultant AnaesthetistDr Michele O’Brien - Consultant AnaesthetistDr Kate Pennington - Consultant AnaesthetistDr Lian Pfitzner - Consultant AnaesthetistDr Kristen Schwager - Consultant AnaesthetistDr Ian Sherratt - Consultant AnaesthetistDr Rasa Venclovas - Consultant AnaesthetistDr Sue Ann Wan - Consultant AnaesthetistDr Harry Wark - Consultant AnaesthetistDr Andrew Weatherall - Consultant AnaesthetistDr Sally Wharton - Consultant AnaesthetistDr Don Hannah - Consultant AnaesthetistDr James MacDonald - Consultant AnaesthetistDr Jane McDonald - Consultant AnaesthetistDr Margaret Perry - Consultant AnaesthetistDr Justin Skowno - Postgraduate Fellow

Australian Paediatric Surveillance Unit (APSU)

StaffProf Elizabeth Elliott - DirectorDr Yvonne Zurynski - Deputy DirectorDr Suwen He - Research Officer EvaluationDr David Lester-Smith - Research OfficerDr Katie Reeve - Research Officer

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Staff and students

Staff and students

Margy Pym - Paediatric Research Co-ordinator Leanne Vidler - Paediatric Research Co-ordinator Dr Deepika Mahajan - Research Fellow Dr Nicola Benwell - Research OfficerNicole McKay - Data Manager Karen Pattinson - Office Co-ordinator Ingrid Charters - Administration Officer Sarah Srikanthan - Publications Project OfficerGreta Ridley - Annual Report Co-ordinatorDr Diana Thomas - Scientific Director, CEBPGAN

StudentsNicola BenwellSuwen HeKate LarkingElizabeth PeadonMichael SmithLouise Tofts

Centre for Kidney Research

Clinical and Laboratory Staff

StaffA/Prof Stephen Alexander - Head of Laboratory ResearchProf Jonathan Craig - Head of Clinical ResearchDr Elisabeth Hodson - Staff Specialist, Nephrology/ Co-Chair Medical Programs, Clinical ProgramsDr David Andresen - Medical MicrobiologistDr Patrina Caldwell - Research Staff SpecialistDenise Campbell - Senior Project OfficerSonia Crampton - Data ManagerDr Anne Durkan - Postgraduate FellowSana Hamilton - Trials Co-ordinatorGail Higgins - Trials Search Co-ordinatorMartin Howell - Research Officer Dr Min Hu - Research ScientistMichelle Irving - Senior Research OfficerRachael Kearns - Research OfficerMarianne Kerr - Trials Recruitment Officer Data ManagerAnh Kieu - Research AssistantPamela Lopez-Vargas - Project OfficerDr Alistair Merrifield - Research FellowRuth Mitchell - Trials Search Co-ordinatorA/Prof Paul Roy - Senior Clinical ResearcherDr Giovanni Strippoli - Honorary Research FellowDr Premala Sureshkumar - Research Fellow

Sean Taylor - Project ManagerDr Allison Tong - Research OfficerEdwina Vale - Data ManagerDr Angela Webster - Senior Research FellowDr Gabrielle Williams - Senior Research OfficerNarelle Williams - Research NurseRita Williams - Senior Aboriginal Health Education OfficerNarelle Willis - Co-ordinator, Cochrane Renal GroupNicola Vukasin - Research OfficerSandra Puckeridge - Office ManagerTamara Borysko - Administration OfficerLeslee Edwards - Administration OfficerDr Yuan Min Wang - Senior Research OfficerDr Debbie Watson - Research AssistantDr Geoff Zhang - Senior Hospital Scientist

StudentsMiriam Codarini - PhD StudentNick Cross - PhD StudentSukanya De - PhD StudentAniruddh Deshpande - PhD StudentJeff Fletcher - PhD StudentHasantha Gunasekera - PhD StudentLeigh Haysom - PhD StudentAngie Morrow - PhD StudentTania Polhill - PhD StudentYashwant Sinha - PhD StudentGermaine Wong - PhD StudentDella Yarnold - PhD Student

Centre for Perinatal Infection Research

StaffDr Marian Fernandez - Postdoctoral ScientistRose White - Senior Research AssistantMaggie Brett - Senior Research Scientist

StudentsEddy Hassan - PhD StudentFranz Puttur - PhD Student

Centre for Trauma Care, Prevention, Education, and Research (CTCPER)

StaffProf Danny Cass - Department Head Dr Lawrence Lam - Scientific Director

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55

Staff and students

Staff and students

Frank Ross - Senior Research AssociatePatricia Manglick - Senior Data ManagerDr Gideon Sandler - Research Fellow

StudentsAinslie MaddockFarah Noaman Kevin Wang

Children’s Hospital Educational Research Institute (CHERI) and Developmental Cognitive Neuropsychology Research Unit (DeCog)

CHERI

Staff Dr Belinda Barton - HeadProf Max Coltheart - Academic Director & James Packer Professor of Educational Research Janelle Levesque - Senior Research AssistantDr Richard Webster - Paediatric Neurologist & ResearcherKatrina Sheraton-Yu - Educator & Intern PsychologistToni Lindsay - Psychologist, Oncology Department

StudentsMichael Gascoigne - PhD Student Ajesh George - PhD Student Susan van den Berg - PhD Student

DeCog

StaffClinical A/Prof Pam Joy - Senior Clinical Neuropsychologist Ruth Brunsdon - Senior Clinical Neuropsychologist Sara Coombes - Clinical Neuropsychologist Signy Wegener - Clinical Neuropsychologist

Honoraries: Melanie Porter - Honorary Clinical Neuropsychologist Louise Parry - Clinical Neuropsychologist Graham C Menzies - Psychologists

Doctoral/PhD Students: Farah BudhaniLauren GillettPeta Minton

Associates: Suzanne Benson - Senior Clinical Psychologist Polly Pickles - Clinical Psychologist Jane Cramsie - Clinical NeuropsychologistTania Malouf - Psychologist

Children’s Hospital Institute Sports Medicine (CHISM)

StaffDr Robert Parker - CHISM Department Head, Senior Exercise PhysiologistDr Carolyn Broderick - CHISM Staff Specialist Paediatric Sports MedicineA/Prof Gary Browne - CHISM Staff Specialist Paediatric Sports MedicineNancy van Doorn - CHISM Exercise PhysiologistDr Damien McKay - Honorary CHISM Research Fellow Paediatric Sports MedicineDr Quang Dao - VMO Orthopaedic Department/CHISM FellowMiriam Dawes - Administration Co-ordinator

The Children’s Hospital at Westmead Clinical School: Discipline of Paediatrics and Child Health

StaffProf Kathryn North - Associate DeanProf Louise Baur - Deputy Associate DeanA/Prof Cheryl Jones - Sub Dean ResearchDr Russell Dale - Sub Dean Postgraduate Studies and Postgraduate Co-ordinatorDr Rachel Skinner - Deputy Postgraduate Co-ordinatorDr Dianne Campbell - Sub Dean EducationDr Meg Phelps - Chair Child and Adolescent Health RotationDr Anne Morris - Masters Co-ordinator Dr Patrina Caldwell - Medical Research/Resident Staff MentorshipDr David Lester-Smith - Academic FellowKaren Scott - Medical Education LecturerA/Prof Andrew Holland - Co-ordinator Surgical Skills TeachingDr SV Soundappan - Senior LecturerDr Nigel Clarke - Deputy Postgraduate Co-ordinatorDr Fiona Fleming - Lecturer (Masters in Medicine - Paediatrics)Dr Stewart Birt - Lecturer (Masters in Medicine - Paediatrics)

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Staff and students

Staff and students

Dr Leanne Mills - Business and Research ManagerLeigh Smith - Executive OfficerDiane Hanlon - Office ManagerVikki Cheetham - Education Support OfficerJessica Bisschop - Administrative Officer (Masters)Denise Yuille - Administrative Officer (Postgraduate)Sandra Harris - Administrative Officer (Postgraduate - coursework)Rita Gudinho - Administrative OfficerAnna Massara - Administrative OfficerSarah Srikanthan - Administrative Officer (to 2008)Louise Powter - Administrative Officer (to 2009)Tanya Owerko - Administrative OfficerLydia Beltran - Administrative Assistant (GMP)Maureen Baker - Administrative Assistant Fran Devasayaham - Administrative AssistantJanica Tse - Finance Officer

Department of Allergy and Immunology

StaffDr Alyson Kakakios - Department Head Dr Dianne Campbell - Staff SpecialistDr Melanie Wong - Staff SpecialistDr Sam Mehr - Research Fellow Dr Paul Turner - Research Fellow

Gene Therapy Research Unit

Children’s Medical Research Institute and The Children’s Hospital at Westmead (Joint Unit)

StaffProf Ian Alexander - Unit HeadDr Sharon Cunningham - Research FellowDr Julie Curtin - Clinical ResearcherDr Samantha Ginn - Noel Dowling Research FellowClaus Hallwirth - Research Fellow Margot Latham - Research AdministratorSophia Liao BSc (Hons) - Research Assistant Grant Logan - Research FellowChristine Smyth - Senior Research OfficerAfroditi Spinoulas - Research AssistantMaolin Zheng - Senior Research Assistant

StudentsAllison Dane - PhD StudentClaire Deakin - PhD StudentCindy Kok - PhD Student

Human Genome Research Program

Genetic Metabolic Disorders Research Group (including the NSW Centre for Rett Syndrome Research)

StaffProf John Christodoulou - Director, Western Sydney Genetics ProgramDr Sarah Williamson - Postdoctoral ScientistDr Wendy Gold - Postdoctoral ScientistDr Xing Zhang Tong - Postdoctoral ScientistGladys Ho - Research Scientist

StudentsRoksana Armani - PhD StudentElisa Bettella - PhD StudentGladys Ho - PhD StudentAbidali Mohamedali - PhD StudentVidya Nelaturi - PhD Student Dr Margaret Perry - PhD StudentKatrina Slater - MPhil Student

Biochemical Genetics and Newborn Screening Research Group

StaffProf Bridget Wilcken - Director, NSW Biochemical Genetics and Newborn Screening ServicesDr Kevin Carpenter - Head, Biochemical GeneticsA/Prof Veronica Wiley - Head, Newborn ScreeningTiffany Wotton - Hospital Scientist

Eye and Developmental Genetics Research Group, Western Sydney Genetics Program, CHW

Eye Genetics Research Group, Embryology Unit, CMRI

StaffDr Robyn Jamieson - Department HeadDr Yongjuan Chen - Sir Norman Gregg Postdoctoral FellowDr Linda Weaving - CJ Martin Postdoctoral Fellow Rebecca Storen - Research Assistant

StudentsRebecca Greenlees - Honours StudentMaja Popovic - Honours StudentLuke St Heaps - Masters StudentWan Yi Ng - PhD Student

Marfan Syndrome Research Group

StaffDr Lesley Adès - Department HeadA/Prof Bruce Bennetts - Head, Department of Molecular Genetics

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57

Staff and students

Staff and students

Dr Andrew Biggin - Honorary Research FellowKatherine J Holman - Hospital Scientist

StudentsKate Sullivan - PhD Student

Skeletal and Lysosomal Disorders Research Group (collaborating with the Centres for Children’s Bone Health and Genetic Innovative Therapies)

StaffProf David Sillence - Department HeadDr Louise Tofts - Fellow in Musculoskeletal RehabilitationDr Lanie Alcausin - Fellow in Medical Genetics (Bone and Mineral Disorders)Dr Michel Tchan - Fellow in Medical Genetics (Genetic Medicine)Kerry Devine - Genetic Therapies Co-ordinatorLynne Foxall - Bisphosphonate Therapies Research Co-ordinator

Institute for Neuromuscular Research/ Neurogenetics Research Unit:

incorporating Neuroimmunology and Neuropathology

StaffProf Kathryn North - Department HeadDr Nan Yang - Team Leader/Senior Hospital ScientistDr Peter Houweling - Research OfficerDr Jane Seto - Research OfficerDr Sandra Cooper - Team Leader/Senior Hospital ScientistJoanne Hawkes - Technical AssistantNigel Clarke - Co-Team Leader/Clinical GeneticistDr Bilijana Ilkovski - Co-Team Leade/Senior Research OfficerDr Jonathan Payne - Team Leader/NeuropsychologistJennifer Lorenzo - Educational PsychologistShelley Robertson - Research AssistantDr Richard Webster - NeurologistNatalie Pride - Research AssistantDr Simone Ardern Holmes - Neurogenetics FellowDr Joshua Burns - Team Leader/Senior Research FellowStephanie Wicks - Clinical Trials EvaluatorDr Emily Oates - Neurogenetics FellowDr Russell Dale - Co-Team Leader/Senior Lecturer/ FellowDr Fabienne Brilot-Turville - Co-Team Leader/Senior Research Officer

Vera Merhab - Research AssistantA/Prof Brian Owler - Team Leader/NeurologistDr Lucy Wang - Research OfficerDr Tom Pitham - Research Fellow

StudentsDr Mimi Berman - PhD StudentPaula Bray - PhD StudentAngela Chen - PhD StudentAlice Ding - Honours StudentDr Ana Domazetovska - MBBS Honours StudentFrances Evesson - PhD StudentDavid Fitzsimons - PhD StudentFleur Garton - PhD StudentMarshall Hogarth - PhD StudentMichaela Kreissl - Overseas Masters StudentMonkol Lek - PhD StudentFrances Lemckert - Masters StudentNancy Mokbel - PhD StudentDr Sekhar Pillai - Masters StudentKristy Rose - PhD Student & Clinical Trials Co-ordinatorDr Esther Tantsis - Masters StudentJenny Tran - MBBS Honours StudentLeigh Waddell - PhD Student/Laboratory Data Co-ordinator

Institute of Endocrinology and Diabetes

Staff

Senior medical research staff: A/Prof Chris Cowell - Director, Institute of Endocrinology and Diabetes A/Prof Kim Donaghue - Head of Diabetes Complications Assessment Service A/Prof Geoffrey R Ambler - Head of Clinical Information and Systems Dr Neville J Howard - Senior Staff Specialist, Principal Investigator, TRIGR (Australia)Dr Maria Craig - Staff Specialist, Conjoint Senior LecturerProf Martin Silink - Senior Staff SpecialistDr Craig Munns - Senior Staff SpecialistDr Shubha Srinivasan - Staff SpeacialistDr Ann Maguire - Staff Specialist

Research Fellows:Dr Sarah Garnett - Clinical Research FellowDr Kim Ramjan - Research FellowDr Paul Benitez-Aguirre - Research FellowDr Myra Poon - Research Fellow

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Staff and students

Staff and students

Research Officers and Research Assistants: Jim Minchenko - Research Scientist, Endocrine LaboratoryCharmaine Tam - PhD CandidateAlison Pryke - Research Assistant, Diabetes Complications & AssessmentRos Bongiorno - TRIGR Nutrition Co-ordinatorGlenda Fraser - CNC TRIGR Nurse Co-ordinatorJanine Cusumano - CNS Diabetes Complications & Assessment Co-ordinatorMargaret Lloyd - CNC Diabetes Prevention Research Nurse Co-ordinatorLynne Foxall - RN Research Trial Co-ordinatorLori Hopley - Nutrition and Dietetics, Research AssistantSukanya de - Research OfficerRebecca Cook - Nutrition and Dietetics, Research Assistant

Clinical research and support staff:Catherine Kay - Business Manager Albert Chan - Clinical Info and Systems Manager/ StatisticianJenny Lee - Manager, Endocrine LaboratoryOksana Markovych - Scientist, Endocrine LaboratoryDarna Bradford - Scientist, Endocrine LaboratoryLiz Lawrie - CNC Endo-testing UnitKelly Winning - RN Endo-testing UnitMary Maquade - CNC Bone Services Nurse Co-ordinatorNuala Harkin - Nurse PracitionerRachel Hayes - Senior Diabetes Dietitian

James Fairfax Institute of Paediatric Nutrition/Gastroenterology

StaffProf Kevin Gaskin - Department HeadDr Caron Blumenthal - Senior Research DietitianCheryl Frazer - Personal Assistant/Admin Officer

StudentsDorothea Stark - PhD Student Sharon Youde - M Sci (Med) Student

Kids Heart Research

StaffA/Prof David Winlaw - Department HeadJody Middlemiss - Administrative Co-ordinatorGillian Blue - Genetic Counsellor

Dr Tanya Butler - Senior ScientistDr Giorgia Esposito - Postdoctoral ScientistJoanne Hawkes - Animal TechnicianGina Walizada - Research Assistant

StudentsCarol Au - PhD StudentTeresa Bonello - Honours StudentDr Jonathan Egan - PhD Student

National Centre for Immunisation Research and Surveillance of Vaccine Preventable Diseases (NCIRS)

StaffProf Peter McIntyre - Director, NCIRSProf Robert Booy - Head, Clinical Research Prof Raina MacIntyre - Senior Principal Research Fellow Dr Kristine Macartney - Deputy Director - Policy Support Robert Menzies - Acting - Surveillance Manager Indigenous/Migrant Health Projects and Program EvaluationDr Jane Jelfs - Manager Policy Support Dr Julie Leask - Manager Social Research Dr Leon Heron - Manager Clinical TrialsDr Glenda Lawrence - Senior Research FellowDr Nicholas Wood - Senior Clinical Research FellowDr Clayton Chiu - Senior Clinical Research FellowDr Yeqin Zuo - Senior Clinical Research FellowDr Gulam Khandaker - Clinical Research FellowDr Danforn Lim - Clinical Research FellowDr Helen Quinn - Research Fellow Brynley Hull - EpidemiologistDr Aditi Dey - EpidemiologistPaula Spokes - EpidemiologistTelphia Joseph - National Indigenous Immunisation Co-ordinator Han Wang - Statistician/Data ManagerDr Sanjay Jayasinghe - Senior Policy OfficerDr Spring Cooper - Senior Research OfficerDr Deepika Mahajan - Senior Research OfficerDr Iman Ridda - Research OfficerMamta Porwal - Research OfficerAmanda Edkins - Research Assistant Swati Ghotane - Research AssistantAnita Heywood - Research Assistant Dr Kevin Yin - Research AssistantMaria Chow - Research Assistant Linda Hueston - Scientific Officer (Laboratory Studies)Ralf Itzwerth - Project Officer

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59

Staff and students

Staff and students

Elizabeth Clarke - Research Nurse Margaret Rose Joyce - Research NurseJennifer Murphy - Research NurseLaura Rost - Research NurseCarol Shineberg - Research NursePamela Cheung - Research NurseElizabeth Deegan - Research NurseEdwina Jacobs - Research NurseCamille Lang - Research NurseMarvin Lester - Research NurseMargaret Pym - PAEDS Co-ordinatorCatherine King - Information ManagerEdward Jacyna - Assistant LibrarianDonna Armstrong - Editing and Publications OfficerDanielle Grant - Communications OfficerKaryn Phillips - Business ManagerLynda Beaumont - Personal AssistantDanielle Marchant - Personal AssistantNicole Jacobs - Personal AssistantJoanne Perkins - Senior Administrative OfficerLyn Benfield - Senior Project Administration Officer

StudentsDr Katherine Hale - PhD StudentTelphia Joseph - MPhilAnthea Katelaris - MBBSCatherine King - PhD StudentSumalathaa Krishnamoorthy - MPH (Hons)Robert Menzies - PhD StudentDr Iman Ridda - PhD StudentDr Nicholas Wood - PhD Student

Nuclear Medicine

StaffProf Robert Howman-Giles - Department HeadDr Kevin London - Honorary Registrar Dr Thomas Pascual - RegistrarA/Prof Vijay Kumar - RadiopharmacyDilip Bodetti - RadiopharmacyJulie Briody - BMD Scientific OfficerTheo Kitsos - Deputy Chief Technologist

Nursing Research & Practice Development Unit

StaffProf Valerie Wilson - DirectorMargaret Kelly - Clinical Nurse ConsultantDr Sarah Redshaw - Senior ResearcherChris White - REACH Project Co-ordinator

Rachel Primrose - Teamwork-Learning-Change/ Essentials of Care Program implementation and Evaluation Co-ordinator

StudentsAmy Barker - Honours ResearchDanielle Doughty - Honours ResearchMargaret Kelly - PhD StudentElaine McCall - Professional DoctoratePippa Mitchell - Masters StudentRuth Mychael - Honours Research

Obesity Research Group

StaffProf Louise Baur - HeadGillian Brown - Administrative OfficerDr Paola Espinel - Project Officer for Australasian Child & Adolescent Obesity Research Network Kelly Kornman - Research Assistant, Loozit® Implementation Project - 2009Anthea Lee - Research Assistant, PEACH Study and Loozit® StudyKristy McGregor - Research Assistant, Loozit® Study - 2008Binh Nguyen - Research Assistant, Loozit® Study - 2009Janice O’Connor - Research Officer, Project Manager, Loozit® Study

StudentsGenevieve Dwyer - PhD StudentVanessa Shrewsbury - PhD StudentCharmaine Tam - PhD Student

Oncology Research Unit

StaffA/Prof Jennifer Byrne - Acting Unit Head and Group LeaderDr Daniel Catchpoole - Group LeaderProf Peter Gunning - Group Leader, Cellular Oncology (to Dec 2008)Dr Belinda Kramer - Group LeaderDr Loretta Lau - Group LeaderDr Geraldine O’Neill - Group LeaderPeta Bradbury - Research AssistantDr Yuyan Chen - Research Officer Dr Albert Chetcuti - Project OfficerJanett Clarkson - Research & Development ManagerLisa Corcoran - Research AssistantRebecca Dagg - Research Assistant

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Staff and students

Staff and students

Vanita D’Souza - Research Support OfficerDr Thomas Fath - Research OfficerDachuan Guo - Research AssistantKim Guven - Research AssistantJayne Hardy - Research AssistantNicholas Ho - Computational Biologist Jeff Hook - Research AssistantJessica Hyman - Research AssistantHelen Jankowski - Research AssistantKerrie Jones - Tumor Bank Database AdministratorDr Antonio Lee - Research OfficerDr Justin Lees - Research OfficerFrances Lemckert - Research AssistantDr Kimberley Lilischkis - Project OfficerDr Christine Lucas - Research OfficerNicole Mackie - Technical OfficerMaha Mahmassani - Research AssistantGuy Nelmes - Research AssistantJuliaka Palmer - Clinical Nurse ConsultantDr Andre Paul - Research OfficerDr Galina Schevzov - Research OfficerRadhika Singh - Quality OfficerDr Justine Stehn - Research OfficerRenee Szokolai - Research AssistantDr Judith Weidenhofer - Research OfficerAdrienne Williams - Research Assistant

StudentsAhmed Al Oqaily - PhD Student (External)Cuc Bach - PhD Student/Research AssistantJamie Baquiran - BmedSc Honours StudentNatalie Berents - BSc Honours StudentTeresa Bontello - BMedSc Honours StudentSara Bontempo - BSc Honours StudentAgnes Chan - Masters StudentAndrea Connor - BSc Honours StudentJason Coombes - PhD StudentLauren Cowell - PhD StudentSarah Creed - PhD StudentNikki Curthoys - PhD StudentAustin Della Franca - PhD StudentSarah Frost - BSc Honours StudentTony Huynh - BSc Honours StudentPrathibha Kahatapitiya - PhD StudentClaire Martin - PhD StudentKarrnan Pathmanandavel - MBBS StudentNatalie Pfund - MBBS StudentBenhur Reynauld - BSc Honours StudentNuruliza Rozlan - PhD Student

Hamideh Shahheydari - PhD StudentMona Shehata - PhD Student/Research AssistantKeerthi Thamotharampillai - PhD StudentFranco Ubaudi - PhD Student (External)Claire Van Der Helm - Dutch Pharmacy StudentSteven Wolf - PhD StudentJessie Zhong - PhD Student/Research Assistant

Orthopaedic Research and Biotechnology Unit

StaffA/Prof David Little - DirectorDr Aaron Schindeler - Research ScientistDr Michelle McDonald - Research OfficerDr Craig Godfrey - Veterinary Surgeon/PhD StudentAlyson Morse - Research AssistantKate Sullivan - Research Assistant Haoi-Lan Mai - Technical Assistant Kathy Mikulec - Technical Assistant Lauren Peacock - Technical AssistantDr Oliver Birke - Orthopaedic Research FellowDr Paul Bokko - Research OfficerDr Mark Latimer - Orthopaedic Research Fellow

StudentsJad El-Hoss - PhD StudentTajana Lah - Honours StudentRenjing Liu - PhD StudentNicole Yu - PhD Student

Psychological Medicine

StaffDr David Dossetor - Area Director for Mental HealthDr Jean Starling - Head of DepartmentDr Sloane Madden - Child PsychiatristDr Kasia Koslowska - Child PsychiatristDr Michael Bowden - Child Psychiatrist Dr Catherine Wiltshire - Child PsychiatristDr Angela Dixon - Senior Clinical PsychologistDr Karen Hancock - Research PsychologistKaren Munro - Senior Clinical PsychologistSiew Koo - Clinical Psychologist Dr Michelle Wong - Clinical Psychologist Belinda Ratcliffe - PsychologistCassandra Hainsworth - Research Psychologist

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Staff and students

Staff and students

Respiratory Medicine Research (RMR)

StaffProf Peter van Asperen - Head of Department and MacIntosh Professor of Respiratory MedicineDr Peter Cooper - Staff Specialist A/Prof Dominic Fitzgerald - Staff SpecialistSamantha Forbes - Research NurseMeredith Larkin - Research NurseMerilyn McArthur - Research NurseDr Karen McKay - Research Fellow and Co-ordinatorTracey Marshall - CNC Asthma EducationAnna Middleton - Research Physiotherapist and PhD StudentMargherita Pitman - Research Support WorkerDr Paul Robinson - Respiratory Fellow and PhD StudentDr Hiran Selvadurai - Staff Specialist

StudentsMichael Ho - USyd MP Honours StudentAlice Kwok - USyd MP Honours Student

Sudden Infant Death Syndrome (SIDS) and Sleep Apnoea Research

StaffA/Prof Karen Waters - HeadDr Rita Machaalani - Post-Doctoral ResearcherChenda Castro - Clinical Research Co-ordinator

StudentsCarla Evans - PhD StudentRebecca Hensley - MPhil StudentDr Joanna MacLean - PhD StudentCourtney Smith - Research StudentSamantha Tang - PhD Student

The Grace Centre for Newborn Care Research Unit

StaffA/Prof Nadia Badawi - Co-Head Grace Centre for Newborn Care and Medical DirectorDr Peter Barr - Senior Staff Specialist Dr Kathryn Browning Carmo - Staff Specialist Dr Robert Halliday - NeonatologistKristen James - Clinical Nurse SpecialistCaroline Karskens - Audit Officer and Research Assistant Dr Alison Loughran - Fowlds Staff Specialist

Jenny McIntosh - Occupational Therapist Rachel Rolinson - Occupational Therapist Kaye Spence - Clinical Nurse ConsultantJan Stewart - Research Nurse

StudentsAmy Barker - BN Honours StudentChristine Jorgenson - BN Honours StudentSharon Laing - PhD StudentSarah McIntyre - PhD StudentMelessa Schultz - BMed Honours StudentKaren Walker - PhD Student

VirologyA/Prof Alison Kesson - Department HeadHanady Elbab - PhD StudentDiane Grote - M Med Student

Wound Healing Laboratory/Children’s Hospital Burns Research Institute (CHBRI)A/Prof Andrew Holland - Director, Children’s Hospital Burns Research InstituteDr John Harvey - Department Head, Burns UnitDr Rachael Murray - Department Head, Wound Healing LaboratoryDr Heather Medbury - Senior Scientist Dr Ann Guiffre - Senior Scientist Dr Monique Bertinetti - Burns Fellow Dr Vasant Rajan - Burns FellowDr Hugh Martin - VMODr Erik La Hei - SurgeonDr Peter Hayward - SurgeonDr John Vandervord - Plastic & Reconstructive SurgeonDr Robert Gates - Plastic Surgeon

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62 Grants

Major Grants awarded in 2008 Calendar Year

Funding source Investigators Title Amount

Australian Orthopaedic Association

A/Prof David Little, Dr Hala Zreiqat

Novel scaffolds for repairing bone defects $58,658

Australian Research Council

Dr Mike Gold, Dr Annette Braunack-Mayer, Prof Phillip Ryan, Prof Christian Gericke, Prof John McNeil, A/Prof Jane Freemantle, Prof Colin Thomson, A/Prof Libby Roughead, Prof Elizabeth Elliott

Can and should we link data at a national level? Vaccine safety surveillance: A case study

$454,232

A/Prof Cheryl Jones, Prof Tony Cunningham, A/Prof Stephen Alexander

The effect of age on regulatory T cell control of the innate and adaptive antiviral immune responses

$318,000

Prof Kathryn North, Dr Gavin Austin Huttley , Prof Gregory J Cooney

Molecular dissection of the effects of alpha-actinin-3 deficiency on normal variation in skeletal muscle function

$432,776

Kaye Spence, A/Prof Nadia Badawi

The emotional and social burden of caring for a young child with complex health needs

$140,291

Prof Robert BooySocial, Economic, and Health Benefits of Vaccinating Children in Day Care Centres against Influenza

$594,265

Australian Rotary Health Research Fund

Prof John Christodoulou, Prof Ian Alexander

Development of Novel Treatment Strategies for Phenylketonuria

$53,745

APEX Foundation Dr Belinda BartonDoes the cognitive psychoscial profile of children with velocardiofacial syndrome (VCFS) differ from an IQ matched control group?

$12,403

Brain Australia Foundation

Dr Russell Dale, Dr Fabienne Brilot

Brain demyelination in children: development of a novel autoantibody method to detect antibodies against cell surface proteins

$38,450

Cancer Council Dr Lyndal Trevena, Dr Julie LeaskRG 08-14 A randomised trial of a web-based toolkit for applying evidence in the general proctice cervical cancer prevention visit

$245,500

Children's Tumor Foundation

Dr Aaron SchindelerModelling the loss of NF1 heterozygosity in congenital pseudarthrosis of the tibia (CTP)

$100,000

Department of Health and Ageing

Prof Elizabeth Elliott, Dr Yvonne Zurynski, Prof Peter McIntyre, Prof Robert Booy

Paediatric Active Enhanced Disease Surveillance (PAEDS)

$136,000

Diabetes Australia Research Trust

Dr Sarah Garnett, A/Prof Chris Cowell, Prof Louise Baur

An RCT examining the effect of two different dietary interventions on insulin resistance in adolescents

$49,160

Dr Amanda Edgely, Prof David Harris, A/Prof Stephen Alexander, A/Prof Darren Kelly

CCL2 Vaccination Protects Against Cardiac Fibrosis

$57,000

Foundation for Children

Prof Louise BaurLoozit®: a community-based healthy, active living program for overweight and obese young people aged 13-16 years

$61,520

Prof Jonathon Craig

ARDAC Second Phase Study - Antecedents of renal disease in Aboriginal and Non- Aboriginal Children and Young adults (a 12 year follow-up study)

$134,684

International Science Linkages

Prof Ian Alexander

A novel approch to the investigation of gene transfer technology-related genotoxicity using junction fragment enrichment and Massiely parallel sequencing: implications for human gene therapy

$487,688

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63 Grants

Major Grants awarded in 2008 Calendar Year

Funding source Investigators Title Amount

Juvenile Diabetes Research Foundation

Prof Tien Wong, A/Prof Kim Donaghue

Prediction of Microvascular Complications using Novel Retinal Imaging

$107,614

Lilly Partnership in Diabetes Research Awards

Dr Maria Craig, Prof William Rawlinson, Dr Neville Howard

Enterovirus infection of pancreatic beta cells using isolates from children with autoimmunity and type 1 diabetes

$40,000

March of Dimes

Prof Kathryn North, Prof Stanley C Froehner 

The Role of Contactin-1 in serve Childhood Myopathy

$US282,585

Prof John Christodoulou, Dr Sandra Cooper

The Utility of forced Myogenesis of cells in culture: Unmasking mitochondrial Respiratory Chain (RC) Disorders

$262,708

MBF FoundationDr Sarah Garnett, A/Prof Chris Cowell, Prof Louise Baur

The role of dietary protein and exercise in pre-diabetic adolescents

$397,975

National Health and Medical Research Council

A/Prof David Little, Dr Paul Baldock, Prof Ian Alexander, Dr Aaron Schindeler

Modelling the loss of NF1 heterozygosity in congenital pseudarthrosis of the tibia (CPT)

$462,500

A/Prof Stephen Alexander, Dr Alex Bishop and Dr Alexandra Sharland

Kidney transplantation: Improving outcomes $427,500

Prof Jonathan Craig, Dr Elizabeth Hodson, Dr David Lyle, Ms Della Yarnold, A/Prof Paul Roy, Dr Leigh Haysom

Antecedents of renal disease in Aboriginal children and young adults – 12 years follow-up

$1,738,125

Dr Geraldine O'Neill, Prof Peter Gunning

Understanding focal adhesion dynmics in cell migration

$258,250

Prof Kathryn North, Dr Edna Hardeman, Prof Peter Gunning, Dr Stewart Head, Dr Nan Yang

The influence of alpha actinins on human performance

$466,875

Dr Rachael MurrayIntegin intracellular transport, expression and function in macrophages regulates inflammation during wound healing

$416,625

Prof Ian Alexander, Dr Alex Bishop

Induction of antigen-specific humoral tolerance by rAAV-mediated delivery of CTLA4-Ig-antigen fusion molecules

$497,250

Prof Peter McIntyreEar health, hearing, speech and language development in Urban Aboriginal children

$430,750

Prof Anne B Chang, Dr Peter Morris, Prof Colin B Robertson, Prof Peter van Asperen , Prof Nicholas J Glasgow, Dr I Brent Masters

Multicentre evaluation of a clinical pathway for chronic cough in children - can its use improve clinical outcomes?

$932,975

Dr Maria CraigViral triggers of autoimmunity and type 1 diabetes: a prospective study of at risk children

$456,000

Prof Tien Wong, A/Prof Kim Donague, A/Prof Alicia Jenkins, Dr Gabriella Tikellis

Prediction of Mircovascular complications in Type 1 diabetes using novel retinal vascular Imaging techniques

$383,325

Dr Genevieve McAuthur, Prof Max Coltheart, Prof Anne Castles

A Randomised control trial of treatments for children with different types of reading difficulty

$840,000

A/Prof Maria Kavallaris, Prof Peter Gunning

Targeting the cytoskeleton in cancer $456,000

Prof Ann Simpson, Dr Bronwyn O'Brien, Prof Ian Alexander

Reversal of diabetes in pigs using liver-directed gene therapy

$550,500

Prof Kathryn North, Dr Sandra Cooper

Biolistic PDS-1000/Hepta Gene Transfer System

$42,997

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64 Grants

Major Grants awarded in 2008 Calendar Year

Funding source Investigators Title Amount

Nurses and Midwives Board NSW

Prof Valerie WilsonEnhancing "Family Centred Care' to improve the health and psychosocial outcomes of hospitalised children

$27,258

Ophthalmic Research Institute of Australia

Dr Robyn JamiesonOcular developmental disorders: molecular genetics and gene function

$50,000

Perpetual Foundation

Prof Kevin Gaskin, Dr Caron Blumenthal

A simple breath test to assess fat absorption in babies with cystic fibrosis

$40,000

Pfizer Australian Research

Dr Kim Ramjan, Dr Sarah Garnett, Dr Craig Munns, A/Prof Chris Cowell

The effect of whole body vibration training on insulin sensitivity in overweight adolescents

$50,000

Ramaciotti Foundation

Prof Kathryn North, Prof John Christodoulou, Prof Peter Gunning, A/Prof Jennifer Byrne

Real-time Amplification System (QPCR) and high resolution Melt (HRM) - Gene Expression and analysis Facility

$30,000

Smile Foundation Prof Kathryn NorthDiagnostic and therapy of Muscular dystrophies and Inherited Myopathies

$100,000

Muscular Dystrophy Association

Dr Sandra Cooper, Prof Kathryn North

Dysferlin and defective muscle membrane repair in limb girdle muscular dystrophy

$64,584

US Army Medical Research and Materiel Command Office of Congressionally Directed Medical Research Programs

Prof Kathryn NorthA Randomized Placebo-Controlled Study of Lovastatin in Children with Neurofibromatosis Type 1

$US2,136,742

US Muscular Dystrophy Association

Prof Stanley C Froehner, Prof Kathryn North

Role of Contactin-1 in servere Congtenital Myopathy and Skeletal Muscle Regulation

$US282,585

University of Sydney

Dr Julie Leask

Collaboration to improve child Health through social research: Understanding and improving under-immunisation in children from disadvantaged population groups

$10,000

Fellowships

Creswick Foundation

Dr Yvonne Zurynski

Rare Childhood Diseases in Australia: Using knowledge and experience from overseas to underpin the development of a national website providing information, education and opportunities for linking families affected by rare diseases in Australia

$15,152

Cancer Institute NSW

Dr Loretta LauSelective targeting of the actin cytoskeleton in cancer therapy

$913,078

INSERM Dr Auralie VanderbrookGene discovery and disease pathogenesis in the inherited myopathies

$ 212,090

National Health and Medical Research Council

Dr Mohamud SheikhInfection, Immunity and immigration – prevention and control of infectious diseases in refugee children

$274,000

Dr Kate QuinlanThe role of growth factors in pluripotency and differentstion of human embryonic stem cells - towards human therapy

$313,847

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65 Our supporters

Major Grants awarded in 2008 Calendar Year

Funding source Investigators Title Amount

Scholarships

Australian Rotary Health Research Fund

Austin Della-FrancaMolecular mechanisms underlying cellular transfermation by D52 overexpression in ovarian cancer

$75,000

National Health and Medical Research Council

Nicole YuManipulating the anabolic and catabolic responses for Bone Tissue engineering

$44,514

Leigh WaddellTo apply evidence-based practice to the diagnosis of muscular dystrophies

$65,598

Anita HeywoodTravel patterns, traveller behaviour and their relationship to the importation of infectious disease into Australia

$43,732

Vanessa ShrewsburyTreatment of overweight and obesity in adolescence

$55,126

Dr Paul RobinsonThe Clinical Utility of Small Airway Function Tests in Paediatric Respiratory Disease

$62,844

The University Of Sydney

Frances EvessonThe role of dysferlin in muscular dystrophy and membrane repair

$60,021

Monkol LekMolecular mechanisms of Alpha-actinin-3 deficiency on skeletal muscle function

$60,021

Angela ChenTrafficking and function of dysferlin: consequences of patient-derived mutations

$60,021

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66

Publications

Publications

2008

› Ades LC (2008). Evolution of the face in Loeys-Dietz syndrome type II: longitudinal observations from infancy in seven cases. Clinical Dysmorphology 17(4): 243-8.

› Ahmed A (2008). MRI features of disseminated ‘drop metastases’. South African Medical Journal 98(7): 522-3.

› Ajayi-Obe EK, Coen PG, Handa R, Hawrami K, Aitken C, McIntosh ED, Booy R (2008). Influenza A and respiratory syncytial virus hospital burden in young children in East London. Epidemiology and Infection 136 (8): 1046-58.

› Al Shanafey S, Harvey J (2008). Long gap esophageal atresia: an Australian experience. Journal of Pediatric Surgery 43(4): 597-601.

› Alexander DM, Hermens DF, Keage HAD, Clark CR, Williams LR, Kohn MR, Clarke SD, Lamb C, Gordon E (2008). Event-related wave activity in the EEG provides new marker of ADHD. Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology 119(1): 163-79.

› Alexander E, Telfer P, Rashid H, Ali KA, Booy R (2008). Nasopharyngeal carriage rate of Streptococcus pneumoniae in children with sickle cell disease before and after the introduction of heptavalent pneumococcal conjugate vaccine. Journal of Infection and Public Health 1(1): 40-44.

› Alexander IE, Cunningham SC, Logan GJ, Christodoulou J (2008). Potential of AAV vectors in the treatment of metabolic disease. Gene Therapy 15(11): 831-9.

› Alexander SI, Smith N, Hu M, Verran D, Shun A, Dorney S, Smith A, Webster B, Shaw PJ, Lammi A, Stormon MO (2008). Chimerism and tolerance in a recipient of a deceased-donor liver transplant. The New England Journal of Medicine 358(4): 369-74.

› Alhusaini A, Crosbie J, Shepherd R, Dean C, Scheinberg A (2008). Passive mechanical properties of the calf muscles in children with cerebral palsy compared to healthy children. Developmental Medicine and Child Neurology 113; 50:38.

› Allen CW, Campbell DE, Kemp AS (2008). Food allergy: Is strict avoidance the only answer? Pediatric Allergy and Immunology: Official Publication of the European Society of Pediatric Allergy and Immunology [Epub].

› Amanata NL, He LH, Swain MV, Little DG (2008). The effect of zoledronic acid on the intrinsic material properties of healing bone: an indentation study. Medical Engineering & Physics 30(7): 843-7.

› Amsili S, Zer H, Hinderlich S, Krause S, Becker-Cohen M, MacArthur DG, North KN, Mitrani-Rosenbaum S (2008). UDP-N-Acetylglucosamine 2-Epimerase/N-Acetylmannosamine Kinase (GNE) Binds to Alpha-Actinin 1: Novel Pathways in Skeletal Muscle? PLoS ONE 3(6):e2477.

› Andre FE, Booy R, Bock HL, Clemens J, Datta SK John TJ, Lee BW, Lolekha S, Peltola H, Ruff TA, Santosham M, Schmitt HJ (2008). Vaccination greatly reduces disease, disability, death and inequity worldwide. Bulletin of the World Health Organization 86(2): 140-6.

› Andrews N, Tischer A, Siedler A, Pebody RG, Barbara C, Cotter S, Duks A, Gacheva N, Bohumir K, Johansen K, Mossong J, Ory F, Prosenc K, Sláciková M, Theeten H, Zarvou M, Pistol A, Bartha K, Cohen D, Backhouse J, Griskevicius A (2008). Towards elimination: measles susceptibility in Australia and 17 European countries. Bulletin of the World Health Organization 86(3): 197-204.

› Aouad LJ, Tam K, Waters KA (2008). Effects of acute intermittent hypercapnic hypoxia on insulin sensitivity in piglets using euglycemic clamp. Metabolism: Clinical and Experimental 57(8): 1056-63.

› Aratchige PE, McIntyre PB, Quinn HE, Gilbert GL (2008). Recent increases in mumps incidence in Australia: the forgotten age group in the 1998 Australian Measles Control Campaign. The Medical Journal of Australia 189(8): 434-7.

› Au C, Butler T, Egan JR, Cooper ST, Lo HP, Compton AG, North KN, Winlaw DS (2008). Changes in skeletal muscle expression of AQP1 and AQP4 in dystrophinopathy and dysferlinopathy patients. Acta Neuropathologica 116(3): 235-46.

› Avery DT, Ma CS, Bryant VL, Santner-Nanan B, Nanan R, Wong M, Fulcher DA, Cook MC, Tangye SG (2008). STAT3 is required for IL-21-induced secretion of IgE from human naive B cells. Blood 112(5):1784-93 [Epub].

› Baoutina A, Alexander IE, Rasko JE, Emslie KR (2008). Developing strategies for detection of gene doping. The Journal of Gene Medicine 10(1): 3-20.

› Barr P, Cacciatore J (2008). Personal fear of death and grief in bereaved mothers. Death. Studies 32(5): 445-60.

› Barth PG, Ryan MM, Webster RI, Aronica E, Kan A, Ramkema M, Jardine P, Poll-The BT (2008). Rhabdomyolysis in pontocerebellar hypoplasia type 2 (PCH-2). Neuromuscular Disorders 18(1): 52-58.

› Bartlett N, Yuan J, Holland AJ, Harvey JG, Martin HC, La Hei AG, Arbuckle S, Godfrey C (2008). Optimal duration of cooling for an acute scald contact burn injury in a porcine model. Journal of Burn Care & Research: Official Publication of the American Burn Association 29(5): 828-34.

› Barzi F, Huxley R, Jamrozik K, Lam TH, Ueshima H, Gu D, Kim HC, Woodward M (2008). Association of smoking and smoking cessation with major causes of mortality in the Asia Pacific Region: the Asia Pacific Cohort Studies Collaboration. Tobacco Control 17(3): 166-72.

› Batty GD, Shipley MJ, Kivimaki M, Barzi F, Smith GD, Mitchell R, Marmot MG, Huxley R (2008). Obesity and overweight in relation to liver disease mortality in men: 38 year follow-up of the original Whitehall study. International Journal of Obesity 32(11): 1741-4.

› Baur LA (2008). Tackling obesity in children and adolescents. BMJ (Clinical research ed.) 337: a1037.

› Begg K, Roche P, Owen R, Liu C, Kaczmarek M, Hii A, Stirzaker S, McDonald A, Fitzsimmons G, McIntyre P, Menzies R, East I, Coleman D, O’Neil K (2008). Australia’s notifiable diseases status, 2006: annual report of the National Notifiable Diseases Surveillance System. Communicable Diseases Intelligence 32(2): 139-207.

› Bell KJL, Irwig L, Craig JC, Macaskill P (2008). Use of randomized trials to decide when to monitor response to new treatment. BMJ 336: 361-5.

› Benitez-Aguirre P, Wood N, Biesheuvel C, Moreira C, Munns C (2008).The natural history of vitamin D deficiency in African refugees living in Sydney. Medical Journal of Australia 190(8):426-8.

› Beutels P, Scuffham PA, MacIntyre CR (2008). Funding of drugs: do vaccines warrant a different approach? Lancet Infectious Diseases 8(11): 727-33.

› Bhate S, Rossiter-Thornton M, Cooper SG, Gillis J, Cole AD, Sholler GS, Chard RB, Winlaw DS (2008). Use of sildenafil and nitric oxide in the management of hypoxemia owing to pulmonary arteriovenous fistulas after total cavopulmonary connection. The Journal of Thoracic and Cardiovascular Surgery 135(2): 446-8.

› Bijarnia S, Wiley V, Carpenter K, Christodoulou J, Ellaway CJ, Wilcken B (2008). Glutaric aciduria type I: outcome following detection by newborn screening. Journal of Inherited Metabolic Disease 31(4): 503-7.

› Branson JA, Dale RC (2008). Transient bilateral blindness and posterior reversible encephalopathy syndrome: a rare complication of enuresis treatment. Journal of Paediatrics and Child Health 44(6): 380-2.

› Brilot F, Jaidaine H, Geenen V, Hober D (2008). Coxsackievirus B4 infection of murine foetal thymus organ cultures. Journal of Medical Virology 80 (4): 659-666.

› Brilot F, Strowig T, Münz C (2008). NK interactions with dendritic cells shape innate and adaptative immunity. Frontiers Bioscience 13: 6443-6454.

› Broderick CR, Herbert RD, Latimer J, Barnes C, Curtin JA, Monagle P (2008). Estimation of transient increases in bleeding associated with physical activity in children with haemophilia. BMC Blood Disorders 8:2.

› Brotherton JM (2008). How much cervical cancer in Australia is vaccine preventable? A meta-analysis. Vaccine 26(2): 250-6.

› Brotherton JM, Deeks SL, Campbell-Lloyd S, Misrachi A, Passaris I, Peterson K, Pitcher H, Scully M, Watson M, Webby R (2008). Interim estimates of human papillomavirus vaccination coverage in the school-based program in Australia. Communicable Diseases Intelligence 32(4): 457-61.

› Brotherton JM, Gold MS (2008). Monitoring vaccine safety: a critical component of every immunisation program. The Medical Journal of Australia 189(5): 243-4.

› Brotherton JM, Gold MS, Kemp AS, McIntyre PB, Burgess MA, Campbell-Lloyd S, New South Wales Health HPV Adverse Events Panel (2008). Anaphylaxis following quadrivalent human papillomavirus vaccination. Canadian Medical Association Journal 179(6): 525-33.

› Browning Carmo K, Terrey A (2008). Stabilising the newborn for transfer - basic principles. Australian Family Physician 37(7): 510-4.

› Browning Carmo KA, Williams K, West M, Berry A (2008). A quality audit of the service delivered by the NSW Neonatal and Paediatric Transport Service. Journal of Paediatrics and Child Health 44(5): 253-72.

› Bruce CR, Hoy AJ, Turner N, Watt MJ, Allen TL, Carpenter K, Cooney GJ, Febbraio MA, Kraegen EW (2008). Over expression of carnitine palmitoyltransferase-1 in skeletal muscle is sufficient to enhance fatty acid oxidation and improve high fat diet-induced insulin resistance. Diabetes [Epub].

› Burns J, Begg L, Vicaretti M (2008). Comparison of orthotic materials on foot pain, comfort, and plantar pressure in the neuroischemic diabetic foot: a case report. Journal of the American Podiatric Medical Association 98(2): 143-8.

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Publications

Publications

› Burns J, Bray P, Cross LA, North KN, Ryan MM, Ouvrier RA (2008). Hand involvement in children with Charcot-Marie-Tooth disease type 1A. Neuromuscular Disorders: NMD 18(12): 970-3.

› Burrell C, Booy R, Wood N, Egan AM, Taverner D, Williams K, Liauw W, Moskwa A, Pepin-Covatta S, Saville M (2008). Immunogenicity of a reduced dose of A/H3N2 in the 2005 southern hemisphere formulation of inactivated split influenza vaccine. Influenza and Other Respiratory Viruses 2(3): 93-8.

› Burrows T, Warren JM, Baur LA, Collins CE (2008). Impact of a child obesity intervention on dietary intake and behaviors. International Journal of Obesity 32(10): 1481-8.

› Butler TL, Egan JR, Graft FG, Au CG, McMahon AC, North KN, Winlaw DS (2008). Dysfunction induced by ischemia versus edema – Does edema matter? Journal of Thoracic and Cardiovascular Surgery 138(1):141-7.

› Cagney M, McIntyre PB, Heron L, Giammanco A, MacIntyre CR (2008). The relationship between pertussis symptomatology, incidence and serology in adolescents. Vaccine 26(44): 5547-53.

› Cairns AG, North KN (2008). Cerebrovascular dysplasia in neurofibromatosis type 1. Journal of Neurology, Neurosurgery, and Psychiatry 79(10): 1165-70.

› Catchpoole DR, Guo D, Jiang H, Biesheuvel C (2008). Predicting outcome in childhood acute lymphoblastic leukaemia using gene expression profiling: Prognostication or protocol selection? Blood 111(4), 2486-2487.

› Cavazzoni E, Schibler A (2008). Monitoring of brain tissue oxygen tension and use of vasopressin after cardiac arrest in a child with catecholamine-induced cardiac arrhythmia. Critical Care and Resuscitation: Journal of the Australasian Academy of Critical Care Medicine 10(4): 316-9.

› Ceely B, Davis A, Hooke N, Kelly M, Lewis P, Watson C (2008). Learning action learning: a journey Action Learning: Research and Practice 5(1), 57-63.

› Chan S, Seto JT, MacArthur DG, Yang N, North KN, Head SI (2008). A gene for speed: contractile properties of isolated whole EDL muscle from an alpha-actinin-3 knockout mouse. American Journal of Physiology. Cell Physiology 295(4): C897-904.

› Chang AC, Hook J, Lemckert F, McDonald MM, Nguyen MA, Hardeman EC, Little DG, Gunning PW, Reddell RR (2008). The murine stanniocalcin 2 gene is a negative regulator of postnatal growth. Endocrinology 149(5): 2403-10.

› Cheng AT, Gazali N (2008). Acquired tracheal diverticulum following repair of tracheo-oesophageal fistula: endoscopic management. International Journal of Pediatric Otorhinolaryngology 72(8): 1269-74.

› Chetcuti A, Adams LJ, Mitchell PB, Schofield PR (2008). Microarray gene expression profiling of mouse brain mRNA in a model of lithium treatment. Psychiatric Genetics 18(2):64-72.

› Cheung N, Rogers SL, Donaghue KC, Jenkins AJ, Tikellis G, Wong TY (2008). Retinal arteriolar dilation predicts retinopathy in adolescents with type 1 diabetes. Diabetes Care 31(9): 1842-6.

› Chiu C, Ridley G, Menzies R, McIntyre P (2008). Update on childhood pneumococcal vaccination. Pediatric Health 2(3): 351-365.

› Cho YH, Taplin C, Mansour A, Howman-Giles R, Hardwick R, Lord D, Howard NJ (2008). Case report: consumptive hypothyroidism consequent to multiple infantile hepatic haemangiomas. Current Opinion in Pediatrics 20(2): 213-5.

› Chow SS, Craig ME, Jones CA, Hall B, Catteau J, Lloyd AR, Rawlinson WD (2008). Differences in amniotic fluid and maternal serum cytokine levels in early midtrimester women without evidence of infection. Cytokine 44(1): 78-84.

› Clarke NF (2008). Skeletal Muscle Disease Due to Mutations in Tropomyosin, Troponin and Cofilin. The Sarcomere and Skeletal Muscle Disease. N. G. Laing. United States, Springer: 40-54. Advances in Experimental Medicine and Biology 642:40-54.

› Clarke NF, Kolski H, Dye DE, Lim E, Smith RL, Patel R, Fahey MC, Bellance R, Romero NB, Johnson ES, Labarre-Vila A, Monnier N, Laing NG, North KN (2008). Mutations in TPM3 are a common cause of congenital fiber type disproportion. Annals of Neurology 63(3): 329-37.

› Cleland V, Crawford D, Baur LA, Hume C, Timperio A, Salmon J (2008). A prospective examination of children’s time spent outdoors, objectively measured physical activity and overweight. International Journal of Obesity 32(11): 1685-93.

› Clement E, Mercuri E, Godfrey C, Smith J, Robb S, Kinali M, Straub V, Bushby K, Manzur A, Talim B, Cowan F, Quinlivan R, Klein A, Longman C, McWilliam R, Topaloglu H, Mein R, Abbs S, North K, Barkovich AJ, Rutherford M, Muntoni F (2008). Brain involvement in muscular dystrophies with defective dystroglycan glycosylation. Annals of Neurology 64(5): 573-82.

› Compton AG, Albrecht DE, Seto JT, Cooper ST, Ilkovski B, Jones KJ, Challis D, Mowat D, Ranscht B, Bahlo M, Froehner SC, North KN (2008). Mutations in contactin-1, a neural adhesion and neuromuscular junction protein, cause a familial form of lethal congenital myopathy. American Journal of Human Genetics 83(6): 714-24.

› Concannon RC, Howman-Giles R, Shun A, Stormon MO (2008). Hepatobiliary scintigraphy for the assessment of biliary strictures after pediatric liver transplantation. Pediatric Transplantation [Epub].

› Cooke A, Deshpande AV, Wong CK, Cohen R (2008). Hepatic derangement following N-Acetylcysteine enemas in an infant with cystic fibrosis. Journal of Paediatrics and Child Health 44(11): 673-5.

› Cooper MG, Corlette T (2008). Dr Corlette and the first textbook of regional anaesthesia in Australia. Anaesthesia and Intensive Care 36 (17): 11.

› Craig JC (2008). Kidney transplantation in children: The preferred option but still no cure. Invited Editorial in American Journal of Kidney Diseases 51(6): 880-1.

› Craig JC, Webster AC, Mitchell A, Irwig L (2008). Expanding the evidence base in transplantation: more and better randomized trials, and extending the value of observational data. Transplantation 86(1):32-5.

› Craig ME, Duffin AC, Gallego PH, Lam A, Cusumano J, Hing S, Donaghue KC (2008). Plantar fascia thickness, a measure of tissue glycation, predicts the development of complications in adolescents with type 1 diabetes. Diabetes Care 31(6): 1201-6.

› Craig ME, Leung KC (2008). Longer-term outcomes of infections in pregnancy: pathogenesis of diabetes and other chronic infections. Microbiology Australia 29(4):186-187.

› Crawford DA, Timperio AF, Salmon JA, Baur L, Giles-Corti B, Roberts RJ, Jackson ML, Andrianopoulos N, Ball K (2008). Neighbourhood fast food outlets and obesity in children and adults: the CLAN Study. International Journal of Pediatric Obesity 3(4): 249-56.

› Creed SJ, Bryce N, Naumanen P, Weinberger R, Lappalainen P, Stehn J, Gunning P (2008). Tropomyosin isoforms define distinct microfilament populations with different drug susceptibility. European Journal of Cell Biology 87(8-9): 709-20.

› Cretikos MA, Valenti L, Britt HC, Baur LA (2008). General practice management of overweight and obesity in children and adolescents in Australia. Medical Care 46(11): 1163-9.

› Crosbie J, Burns J, Ouvrier RA (2008). Pressure characteristics in painful pes cavus feet resulting from Charcot-Marie-Tooth disease. Gait & Posture 28(4): 545-51.

› Cunningham AL, Breuer J, Dwyer DE, Gronow DW, Helme RD, Litt JC, Levin MJ, MacIntyre CR (2008). The prevention and management of herpes zoster. Medical Journal of Australia 188(3): 171-6.

› Cunningham SC, Dane AP, Spinoulas A, Logan GJ, Alexander IE (2008). Gene delivery to the juvenile mouse liver using AAV2/8 vectors. Molecular Therapy: The Journal of the American Society of Gene Therapy 16(6): 1081-8.

› Curtin JA, Dane AP, Swanson A, Alexander IE, Ginn SL (2008). Bidirectional promoter interference between two widely used internal heterologous promoters in a late-generation lentiviral construct. Gene Therapy 15(5): 384-90.

› Dale RC (2008). Acute disseminated encephalomyelitis: where does it start and where does it stop? Developmental Medicine and Child Neurology 50(5): 326-7.

› Dale RC, Brilot F, Fagan E, Earl J (2008). CSF neopterin in paediatric neurology: a marker of active CNS inflammation. Developmental Medicine and Child Neurology 51(4):317-23.

› Daniel A, St Heaps L, Sylvester D, Diaz S, Peters G (2008). Two mosaic terminal inverted duplications arising post-zygotically: Evidence for possible formation of neo-telomeres. Cell & Chromosome [Epub] 7: 1.

› de Brouwer AP, Williams KL, Duley AJ, van Kuilenburg AB, Nabuurs SB, Egmont-Petersen M, Lugtenberg D, Zoetekouw L, Banning MJ, Roeffen M, Hamel BC, Weaving L, Ouvrier RA, Donald JA, Wevers RA, Christodoulou J, van Bokhoven H (2008). Arts Syndrome. Gene Reviews. American Journal of Human Genetics 81(3):507-18.

› De S, Small J, Baur LA (2008). Overweight and obesity among children with developmental disabilities. Journal of Intellectual & Developmental Disability 33(1): 43-7.

› Denney-Wilson E, Hardy LL, Dobbins T, Okely AD, Baur LA (2008). Body mass index, waist circumference, and chronic disease risk factors in Australian adolescents. Archives of Pediatrics & Adolescent Medicine 162(6): 566-73.

› Deshpande AV, La Hei ER (2008). Impact of laparoscopy on the management of transverse testicular ectopia. Journal of Laparoendoscopic & Advanced Surgical Techniques. Part A 19(3): 443-6.

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68

Publications

Publications

› Deshpande AV, Shun A (2008). Laparoscopic treatment of esophageal stenosis due to tracheobronchial remnant in a child. Journal of Laparoendoscopic & Advanced Surgical Techniques. Part A 19(1): 107-9.

› Devictor D, Tissieres P, Gillis J, Truoq R, WFPICCS Task Force on Ethics (2008). Intercontinental differences in end-of-life attitudes in the pediatric intensive care unit: results of a worldwide survey. Pediatric Critical Care Medicine: A Journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies 9(6): 560-6.

› Donaghue K, Mohsin F, Stone M (2008). Chronic Complications of Childhood Diabetes. Epidemiology of Pediatric and Adolescent Diabetes. D. Dabelea and G. J. Klingersmith. United States, Informa Healthcare: 217-233.

› Doust J, Craig JC (2008). Evaluating diagnostic tests - should the same methods apply? American Heart Journal 156: 4-6.

› Duncan CE, Chetcuti AF, Schofield PR (2008). Coregulation of genes in the mouse brain following treatment with clozapine, haloperidol, or olanzapine implicates altered potassium channel subunit expression in the mechanism of antipsychotic drug action. Psychiatric Genetics 18(5):226-39.

› Dwyer GM, Higgs J, Hardy LL, Baur LA (2008). What do parents and preschool staff tell us about young children’s physical activity: a qualitative study. The International Journal of Behavioral Nutrition and Physical Activity [Epub] 5: 66.

› Egan JR, Butler TL, Cole AD, Abraham S, Murala J, Baines D, Street N, Thompson L, Biecker O, Dittmer J, Cooper S, Au CG, North KN, Winlaw DS (2008). Myocardial membrane injury in paediatric cardiac surgery – an animal model. Journal of Thoracic and Cardiovascular Surgery 136 1265-73, 1273 e1-2.

› Egan JR, Butler TL, Cole AD, Aharonyan A, Barnes D, Street N, Navaratnam M, Biecker O, Zazulak C, Au CG, Tan YM, North KN, Winlaw DS (2008). Myocardial ischemia is more important than the effects of cardiopulmonary bypass on myocardial water handling and postoperative dysfunction: A paediatric animal model. The Journal of Thoracic and Cardiovascular Surgery 136(5): 1265-1273.e2.

› Elliott E (2008). Oral rehydration therapy: applied physiology. Clinical Medicine (London, England) 8(3): 296-7.

› Elliott EJ, Bower C (2008). Alcohol and pregnancy: the pivotal role of the obstetrician. The Australian & New Zealand Journal of Obstetrics & Gynaecology 48(3): 236-9.

› Elliott EJ, Payne J, Morris A, Haan E, Bower C (2008). Fetal alcohol syndrome: a prospective national surveillance study. Archives of Disease in Childhood 93(9): 732-7.

› Faivre L, Collod-Beroud G, Child A, Callewaert B, Loeys BL, Binquet C, Gautier E, Arbustini E, Mayer K, Arslan-Kirchner M, Stheneur C, Kiotsekoglou A, Comeglio P, Marziliano N, Halliday D, Beroud C, Bonithon-Kopp C, Claustres M, Plauchu H, Robinson PN, Adès L, De Backer J, Coucke P, Francke U, De Paepe A, Boileau C, Jondeau G (2008). Contribution of molecular analyses in diagnosing Marfan syndrome and type I fibrillinopathies: an international study of 1009 probands. Journal of Medical Genetics 45(6): 384-390.

› Fehon R, Mehr S, La Hei E, Isaacs D, Wong M (2008). Two-year-old boy with cervical and liver abscesses. Journal of Paediatrics and Child Health 44(11): 670-2.

› Fernandez MA, Evans IA, Hassan EH, Carbone FR, Jones CA (2008). Neonatal CD8+ T cells are slow to develop into lytic effectors after HSV infection in vivo. European Journal of Immunology 38(1): 102-13.

› Fernandez MA, Puttur FK, Wang YM, Howden W, Alexander SI, Jones CA (2008). T regulatory cells contribute to the attenuated primary CD8+ and CD4+ T cell responses to herpes simplex virus type 2 in neonatal mice. Journal of Immunology (Baltimore, Md: 1950) 180(3): 1556-64.

› Fitzgerald DA, Massie RJ, Nixon GM, Jaffe A, Wilson A, Landau LI, Twiss J, Smith G, Wainwright C, Harris M (2008). Infants with chronic neonatal lung disease: recommendations for the use of home oxygen therapy. The Medical Journal of Australia 189(10): 578-82.

› Fletcher JT, Nankivell BJ, Alexander SI (2008). Chronic allograft nephropathy. Pediatric Nephrology (Berlin, Germany) 24(8): 1465-71.

› Forrestor M, Mihrshahi S, Waugh MC, O’Flaherty S, Rice J, Graham HK, Scheinberg A (2008). Conscious sedation and general anaesthetic for intramuscular Botulinum toxin-A injections for spasticity management – a two centre cross sectional prospective audit. Developmental Medicine and Child Neurology 113; 50:5.

› Foster J, Bidewell J, Buckmaster A, Spence K, Green D, Henderson-Smart D (2008). Comparison of the Effects of Continuous Positive Airways Pressure and Headbox Oxygen on Nurses’ Workload in the Special Care Nursery. Neonatal, Paediatric and Child Health Nursing 11, 2:11-17.

› Fremantle E, Zurynski YA, Mahajan D, D’Antoine H, Elliott EJ (2008). Indigenous child health: urgent need for improved data to underpin better health outcomes. The Medical Journal of Australia 188(10): 588-591.

› Gabbett MT, Peters GB, Carmichael JM, Darmanian AP, Collins FA (2008). Prader-Willi syndrome phenocopy due to duplication of Xq21.1-q21.31, with array CGH of the critical region. Clinical Genetics 73(4): 353-9.

› Gallego PH, Craig ME, Duffin AC, Bennetts B, Jenkins AJ, Hofer S, Lam A, Donaghue KC (2008). Association between p.Leu54Met polymorphism at the paraoxonase-1 gene and plantar fascia thickness in young subjects with type 1 diabetes. Diabetes Care 31(8): 1585-9.

› Gallego PH, Craig ME, Hing S, Donaghue KC (2008). Role of blood pressure in development of early retinopathy in adolescents with type 1 diabetes: prospective cohort study. BMJ 337: a918.

› Garland SM, Brotherton JM, Skinner SR, Pitts M, Saville M, Mola G, Jones RW (2008). Human papillomavirus and cervical cancer in Australasia and Oceania: risk-factors, epidemiology and prevention. Vaccine 26 (12): M80-8.

› Garnett SP, Baur LA, Cowell CT (2008). Waist-to-height ratio: a simple option for determining excess central adiposity in young people. International Journal of Obesity 32(6): 1028-30.

› Gaskin K, Kleinman RE, Sanderson IR, Goulet O, Sherman PM, Mieli-Vergani G, Shneider BL (2008). Cystic Fibrosis, Walker’s Pediatric Gastrointestinal Disease 45.1; 1227-1239.

› Gaskin KJ, Allen JR, Duggan C, Watkins JB, Walker WA (2008). Exocrine pancreatic disease including cystic fibrosis. Nutrition in Pediatrics 52; 577-588.

› George A, Vickers MH, Wilkes L, Barton B (2008). Working and caring for a child with chronic illness: Challenges in maintaining employment. Employee Responsibilities and Rights Journal 20(3):165-176.

› George A, Vickers MH, Wilkes L, Barton B (2008). Working and caring for a child with chronic illness: Barriers in achieving work-family balance. Journal of Management and Organization 14(1):59-72.

› Giles ML, McDonald AM, Elliott EJ, Ziegler JB, Hellard ME, Lewin SR, Kaldor JM (2008). Variable uptake of recommended interventions to reduce mother-to-child transmission of HIV in Australia, 1982-2005. The Medical Journal of Australia 189(3): 151-4.

› Gillis J (2008). We want everything done. Archives of Disease in Childhood 93(3): 192-3.

› Gleeson H, Wiltshire E, Briody J, Hall J, Chaitow J, Sillence D, Cowell C, Munns C (2008). Childhood chronic recurrent multifocal osteomyelitis: pamidronate therapy decreases pain and improves vertebral shape. The Journal of Rheumatology 35(4): 707-12.

› Gleeson HK, Wiley V, Wilcken B, Elliott E, Cowell C, Thonsett M, Byrne G, Ambler G (2008). Two-year pilot study of newborn screening for congenital adrenal hyperlasia in New South Wales compared with nationwide case surveillance in Australia. Journal of Paediatrics and Child Health 44(10): 554-9.

› Gomes L, Mackie N, Catchpoole DR, Henwood T (2008). Test and Teach – Ina fix about immunohistochemistry on 60 year old tissue blocks? Journal of Histotechnology 8, 125.

› Gubbay JB, Gosbell IB, Barbagiannakos T, Vickery AM, Mercer JL, Watson M (2008). Clinical features, epidemiology, antimicrobial resistance, and exotoxin genes (including that of Panton-Valentine leukocidin) of gentamicin-susceptible methicillin-resistant Staphylococcus aureus (GS-MRSA) isolated at a paediatric teaching hospital in New South Wales, Australia. Pathology 40(1): 64-71.

› Gunasekera H (2008). How to treat: otitis media. Australian Doctor 33-40.

› Gunja N, Doyle E, Carpenter K, Chan OT, Gilmore S, Browne G, Graudins A (2008). Gamma-hydroxybutyrate poisoning from toy beads. The Medical Journal of Australia 188(1): 54-5.

› Gunning P, O’Neill G, Hardeman E (2008). Tropomyosin-based regulation of the actin cytoskeleton in time and space. Physiological Reviews 88(1): 1-35.

› Gunstad J, Spitznagel MB, Paul RH, Cohen RA, Kohn M, Luyster FS, Clark R, Williams LM, Gordon E (2008). Body mass index and neuropsychological function in healthy children and adolescents. Appetite 50(2-3): 246-51.

› Gurer C, Strowig T, Brilot F, Pack M, Trumpfeller C, Array F, Park CG, Steinman RM, Münz C (2008). Targetting the nuclear antigen 1 of Epstein Barr virus to the human endocytic receptor DEC-205 stimulates protective T-cell responses. Blood 112: 1231-9.

› Guyatt GH, Oxman AD, Kunz R, Jaeschke R, Helfand M, Liberati A, Vist GE, Schunemann HJ (2008). GRADE: Incorporating considerations of resources use into grading recommendations. BMJ 336(7654): 1170-3.

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Publications

› Haas V, Kohn MR, Gaskin K, Clarke S, Allen J, Madden S, Lochs H, and Müller MJ (2008). Proteinstatus anorektischer Patientinnen vor und nach Gewichtszunahme. Conference proceedings: Aktuelle Ernährungsmedizin 3:145.

› Haas VK, Kohn MR, Clarke S, Gaskin K, Stark D, and Müller MJ (2008). Regional fat distribution in adolescent anorexic patients before and after medium and long term weight gain. Conference proceedings: International Journal of Obesity 32(S1): S89).

› Haas VK, Kohn MR, Clarke SD, Müller MJ, Gaskin KG (2008). Protein depletion in adolescent females with anorexia nervosa assessed by in-vivo neutron activation analysis. Conference proceedings: International Journal of Body Composition Research 6(2):68.

› Hackett A, Gillard J, Wilcken B (2008). n of 1 trial for an ornithine transcarbamylase deficiency carrier. Molecular Genetics and Metabolism 94(2): 157-61.

› Hang KW, Colville D, Tai R, Jones A, Alexander SI, Fletcher J, Savige J (2008). The use of ocularabnormalities to diagnose X-linked Alport syndrome in Children. Pediatric Nephrology 23(8):1245-50.

› Hansen MA, da Cruz MJ, Owler BK (2008). Endoscopic transnasal decompression for management of basilar invagination in osteogenesis imperfecta. Journal of Neurosurgery. Spine 9(4): 354-7.

› Hawke F, Burns J, Radford JA, du Toit V (2008). Custom-made foot orthoses for the treatment of foot pain. Cochrane Database of Systematic Reviews 0(3): 135.

› Hayden S, Scheinberg A, Ward A (2008). Intrathecal Baclofen for children with spasticity and/or dystonia. Goal attainment and adverse events. Developmental Medicine and Child Neurology 113;50:6.

› Ho G, Walter JH, Christodoulou J (2008). Costeff optic atrophy syndrome: New clinical case and novel molecular findings. Journal of Inherited Metabolic Disease [Epub].

› Ho Shon IA, Chung D, Saw RP, Thompson JF (2008). Guidelines for imaging in cutaneous melanoma. Nuclear Medicine Communications 29(10): 877-879.

› Ho Shon IA, Chung D, Saw RP, Thompson JF (2008). Imaging in cutaneous melanoma. Nuclear Medicine Communications 29(10): 847-876.

› Hodson EM, Alexander SI (2008). Evaluation and management of steroid-sensitive nephrotic syndrome. Current Opinion in Pediatrics 20(2): 145-50.

› Hodson EM, Craig JC (2008). Therapies for steroid-resistant nephrotic syndrome. Pediatric Nephrology 23(9): 1391-4 [Epub].

› Högler W, Blimkie C, Rauch F, Woodhead H, Cowell C (2008). Scaling and adjusting growth-related data and sex-differences in the muscle-bone relation: a perspective. Journal of Musculoskeletal & Neuronal Interactions 8(1): 25-8.

› Högler W, Blimkie CJ, Cowell CT, Inglis D, Rauch F, Kemp AF, Wiebe P, Duncan CS, Farpour-Lambert N, Woodhead HJ (2008). Sex-specific developmental changes in muscle size and bone geometry at the femoral shaft. Bone 42(5): 982-9.

› Holland AJ (2008). A child with recurrent abdominal pain. Journal of Pediatric Surgery 43:1415.

› Holland AJ (2008). Blunt chest trauma in childhood. Journal of Pediatric Surgery 43:955-6.

› Holland AJ (2008). Management of pediatric retropharyngeal infections: nonsurgical vssurgical. Journal of Pediatric Surgery 43:957.

› Holland AJ (2008). Neurotrauma in children. Journal of Pediatric Surgery 43:956.

› Holland AJ (2008). Paraumbilical block for umbilical herniorrhaphy. Journal of Pediatric Surgery 43:959.

› Holland AJ (2008). Rural surgical training in Australia and update: rural and remote surgery. Australian and New Zealand Journal of Surgery 78(7): 619-620.

› Holland AJ, Abubacker M, Smith GH, Cass DT (2008). Management of urethrocutaneous fistula following hypospadias repair. Pediatric Surgery International 24(9): 1047-51.

› Holland AJ, Soundappan SV, Cass DT (2008). Comment on: Missed injury and the tertiary trauma survey [Injury 39:107-114]. Injury 40(1): 110.

› Holland AJ, Soundappan SV, Oldmeadow W (2008). Videoconferencing surgical tutorials: bridging the gap. ANZ Journal of Surgery 78(4): 297-301.

› Holland AJA, Tarran SLS, Medbury HJ, Guiffre AK (2008). Are fibrocytes present in paediatric burn wounds? ANZ Journal of Surgery 78(s 1): A10.

› Holland AL, Tarran SJ, Medbury HJ, Guiffre AK (2008). Are fibrocytes present in pediatric burn wounds? Journal of Burn Care & Research: Official Publication of the American Burn Association 29(4): 619-26.

› Holland D, Booy R, De Looze F, Eizenberg P, McDonald J, Karrasch J, McKeirnan M, Salem H, Mills G, Reid J, Weber F, Saville M (2008). Intradermal influenza vaccine administered using a new microinjection system produces superior immunogenicity in elderly adults: a randomized controlled trial. The Journal of Infectious Diseases 198(5): 650-8.

› Honey A, Boughtwood D, Clarke S, Halse C, Kohn M, Madden S (2008). Support for parents of children with anorexia: what parents want. Eating Disorders 16(1): 40-51.

› Hooke N, Lewis P, Kelly M, Wilson V, Jones S (2008). Making something of it: one ward’s application of evidence into practice. Practice Development in Health Care 7(2), 79-91.

› Horst M, Smith GH (2008). Pelvi-ureteric junction obstruction in duplex kidneys. BJU International 101(12): 1580-4.

› Howman-Giles R, Holland AJ, Mihm D, Montfort JM, Arbuckle S, Kellie S (2008). Somatic malignant transformation in a sacrococcygeal teratoma in a child and the use of F18FDG PET imaging. Pediatric Surgery International 24(4): 475-8.

› Howman-Giles R, London K, McCowage G, Graf N, Harvey J (2008). Pulmonary inflammatory myofibroblastic tumor after Hodgkin’s lymphoma and application of PET imaging. Pediatric Surgery International 24(8): 947-51.

› Hsu B, Cree AK, Lagopoulos J, Cummine JL (2008). Transcranial motor-evoked potentials combined with response recording through compound muscle action potential as the sole modality of spinal cord monitoring in spinal deformity surgery. Spine 33(10): 1100-6.

› Hu M, Watson D, Zhang GY, Graf N, Wang YM, Sartor M, Howden B, Fletcher J, Alexander SI (2008). Long term cardiac allograft survival across an MHC mismatch after pruning of alloreactive CD4 T cells. Journal of Immunology (Baltimore, Md: 1950) 180(10): 6593-603.

› Hu W, Grbich C, Kemp A (2008). When doctors disagree: a qualitative study of doctors’ and parents’ views on the risks of childhood food allergy. Health Expectations: An International Journal of Public Participation in Health Care and Health Policy 11(3): 208-19.

› Hu W, Katelaris CH, Kemp AS (2008). Allergic rhinitis - practical management strategies. Australian Family Physician 37(4): 214-20.

› Hu W, Loblay R, Ziegler J, Kemp A (2008). Attributes and views of families with food allergic children recruited from allergy clinics and from a consumer organization. Pediatric Allergy and Immunology: Official Publication of the European Society of Pediatric Allergy and Immunology 19(3): 264-9.

› Hull BP, Deeks S, Menzies R, McIntyre PB (2008). What do we know about 7vPCV coverage in Aboriginal and Torres Strait Islander children? A 2007 update. Communicable Diseases Intelligence 32(2): 257-60.

› Hunter C, Spence K, McKenna K, Iedema R (2008). Learning how we learn: an ethnographic study of neonatal intensive care. Journal of Advanced Nursing 62, 6:657-664.

› Hunter C, Spence K, Scheinberg A (2008). Untangling the web of critical incidents: Ethnography in a paediatric setting. Anthropology and Medicine 15(2): 91-103.

› Huxley R, Barzi F, Lee CMY, Janus E, Lam TH, Caterson I, Azizi F, Lear S, Patel J, Shaw J, Adam J, Oh SW, Kang JH, Zimmet P, Woodward M (2008). Is central obesity a better discriminator of the risk of hypertension than body mass index in ethnically diverse populations? Journal of Hypertension 26: 169-77.

› Huxley R, James WP, Barzi F, Patel JV, Lear SA, Suriyawongpaisal P, Janus E, Caterson I, Zimmet P, Prabhakaran D, Reddy S, Woodward M, Obesity in Asia Collaboration (2008). Ethnic comparisons of the cross-sectional relationships between measures of body size with diabetes and hypertension. Obesity Reviews: An Official Journal of the International Association for the Study of Obesity 9 (1): 53-61.

› Ilkovski B (2008). Investigations into the pathobiology of thin-filament myopathies. Advances in Experimental Medicine and Biology 64255-65.

› Ilkovski B, Mokbel N, Lewis RA, Walker K, Nowak KJ, Domazetovska A, Laing NG, Fowler VM, North KN, Cooper ST (2008). Disease severity and thin filament regulation in M9R TPM3 nemaline myopathy. Journal of Neuropathology and Experimental Neurology 67(9): 867-77.

› Inglis E, Kesson A, Newall AT, Macartney K, Macintyre CR (2008). The burden of rotavirus gastroenteritis in children presenting to a paediatric hospital. Epidemiology and Infection 137(7): 943-9.

› Isaacs D (2008). Fungal prophylaxis in very low birth weight neonates: nystatin, fluconazole or nothing? Current Opinion in Infectious Diseases 21(3): 246-50.

› Isaacs D (2008). On Fellowship of the Royal Australasian College of Physicians written exam. Journal of Paediatrics and Child Health 44(10): 533.

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› Isaacs D (2008). Commentary: Controversies in SIGN guidance on management of invasive meningococcal disease in children and young people. BMJ (Clinical research ed.) 336(7657): 1370-1.

› Isaacs D, Isaacs S, Fitzgerald D (2008). The seven ages of man and woman (or Sex and the CT). Archives of Disease in Childhood 93(12): 1075-6.

› Isaacs D, Kilham H, Leask J, Tobin B (2008). Ethical issues in immunisation. Vaccine 27(5): 615-8.

› Isaacs D, Kilham HA, Jacobe S, Ryan MM, Tobin B (2008). Gaining consent for publication in difficult cases involving children. BMJ 337a1231.

› Isaacs D, Leask J (2008). Should influenza immunisation be mandatory for healthcare workers? No. BMJ (Clinical research ed.) 337: a2140.

› Isbister GK, Burns J, Prior F, Ouvrier RA (2008). Safety of nitrous oxide administration in patients with Charcot-Marie-Tooth disease. Journal of the Neurological Sciences 268(1-2): 160-2.

› James A, Green F, Abrahamson M, Bai T, Dolhnikoff M, Maud T, McKay K, Elliot J (2008). Airway basement membrane perimeter distensibility and airway smooth muscle area in asthma. Journal of Applied Physiology 104: 1703-1708.

› Jaques A, Daviskas E, Turton JA, McKay K, Cooper P, Stirling RG, Robertson CF, Bye PT, Lesouëf PN, Shadbolt B, Anderson SD, Charlton B (2008). Inhaled mannitol improves lung function in cystic fibrosis. Chest 133(6): 1388-96.

› Jiwane A, Holland AJ, Soundappan S, Arnold J (2008). Letters to the editor. Journal of Paediatrics and Child Health 44(12): 747-9.

› Jiwane A, Holland AJA, Soundappan SVS, Arnold J (2008). An unusual case of bilious vomiting in a neonate. Journal of Paediatric and Child Health 44;747-9.

› Johnston O, Rose CL, Webster AC, Gill JS (2008). Sirolimus is associated with new-onset diabetes in kidney transplant recipients. Journal of the American Society of Nephrology 19(7):1411-8.

› Jones CA (2008). Diagnosis and Treatment of herpes simplex virus infection in the newborn. Microbiology Australia 29 (4) 194-6.

› Jones VS, Biesheuvel CJ, Cohen RC (2008). Impact of minimally invasive surgery on the pediatric surgical profession. Journal of Laparoendoscopic & Advanced Surgical Techniques. Part A 18(6): 881-6.

› Jones VS, Chennapragada MS, Lord DJ, Stormon M, Shun A (2008). Post-liver transplant mycotic aneurysm of the hepatic artery. Journal of Pediatric Surgery 43(3): 555-8.

› Jones VS, Cohen RC (2008). Two decades of minimally invasive pediatric surgery-taking stock. Journal of Pediatric Surgery 43(9): 1653-9.

› Jones VS, Cohen RC (2008). What causes intrathoracic gastric dilatation in a late-presenting diaphragmatic hernia? Pediatric Emergency Care 24(5): 317-8.

› Jones VS, Holland AJ, Oldmeadow W (2008). Inductive teaching method-an alternate method for small group learning. Medical Teacher 30(8): e246-9.

› Jones VS, Pitkin J (2008). Navigating the thoracic inlet in children. Pediatric Surgery International 24(4): 491-4.

› Jones VS, Thomas G, Stormon M, Shun A (2008). The ping-pong ball as a surgical aid in liver transplantation. Journal of Pediatric Surgery 43(9): 1745-8.

› Jones VS, Wood JG, Godfrey C, Cohen RC (2008). An optimum animal model for neonatal thoracoscopy. Journal of Laparoendoscopic & Advanced Surgical Techniques. Part A 18(5): 759-62.

› Kaplan FS, Xu M, Glaser DL, Collins F, Connor M, Kitterman J, Sillence D, Zackai E, Ravitsky V, Zasloff M, Ganguly A, Shore EM (2008). Early diagnosis of fibrodysplasia ossificans progressiva. Pediatrics 121(5): e1295-300.

› Katzmarzyk PT, Baur LA, Blair SN, Lambert EV, Oppert JM, Riddoch C (2008). International conference on physical activity and obesity in children: summary statement and recommendations. Applied Physiology Nutrition and Metabolism-Physiologie Appliquee Nutrition Et Metabolisme 33(2): 371-88.

› Kellie SJ, Al Lamki Z, Agarwal BR, Wali Y, Ngcamu N, Al Sawafi N, Kurkure P (2008). Childhood cancer: quest for a complete cure. Pediatric Blood & Cancer 51(6): 843-5.

› Kellie SJ, Howard SC (2008). Global child health priorities: what role for paediatric oncologists? European Journal of Cancer 44(16): 2388-96.

› Kelly H, Newall AT (2008). Mortality benefits of influenza vaccination in elderly people [letter]. The Lancet Infectious Diseases 8(8): 462-3.

› Kemp A, Ponsonby AL, Dwyer T, Cochrane J, Pezic A, Carmichael A, Carlin J, Jones G (2008). The interaction between early life upper respiratory tract infection and birth during the pollen season on rye-sensitized hay fever and ryegrass sensitization - a birth cohort study. Pediatric Allergy and Immunology: Official Publication of the European Society of Pediatric Allergy and Immunology [Epub].

› Kemp A, Hu W (2008). Food allergy and anaphylaxis - dealing with uncertainty. The Medical Journal of Australia 188(9): 503-4.

› Kemp AS (2008). Allergy and gastric acid suppression. Clinical and Experimental Allergy: Journal of the British Society for Allergy and Clinical Immunology 39(2): 176-8.

› Kemp AS, Hill DJ, Allen KJ, Anderson K, Davidson GP, Day AS, Heine RG, Peake JE, Prescott SL, Shugg AW, Sinn JK (2008). Guidelines for the use of infant formulas to treat cows milk protein allergy: an Australian consensus panel opinion. The Medical Journal of Australia 188(2): 109-12.

› Kemp AS, Hill DJ, Allen KJ, Anderson K, Davidson GP, Day AS, Heine RG, Peake JE, Prescott SL, Shugg AW, Sinn JK, Australian consensus panel (2008). Guidelines for the use of infant formulas to treat cows milk protein allergy: an Australian consensus panel opinion. The Medical Journal of Australia 188(2): 109-12.

› Kesson A, Andresen D, Holt L, Fernando R (2008). Antimicrobial Stewardship: It’s Role in a Tertiary Paediatric Hospital. The Bulletin of the Sri Lankan College of Microbiologists 6(0): 49-54.

› Khan NI, Cisterne A, Devidas M, Shuster J, Hunger SP, Shaw PJ, Bradstock KF, Bendall LJ (2008). Expression of CD44, but not CD44v6, predicts relapse in children with B cell progenitor acute lymphoblastic leukemia lacking adverse or favorable genetics. Leukemia & Lymphoma 49(4): 710-8.

› Khatami A, Singh-Grewal D, Kilham H (2008). Tendon sheath abscess and severe carbamazepine hypersensitivity. Journal of Paediatrics and Child Health 44(7-8): 462-3.

› Khong P, Pitham T, Owler B (2008). Isolated neurolymphomatosis of the cauda equina and filum terminale: case report. Spine 1;33(21):E807-11.

› Kiely CJ, Holland AJ, Cole AD, Winlaw DS, Sholler GF (2008). Transposition of the Great Arteries, Interrupted Inferior Vena Cava and Intestinal Atresia: A New Association? Heart, Lung & Circulation [Epub].

› Kim A, Ji L, Balmaceda C, Diez B, Kellie SJ, Dunkel IJ, Gardner SL, Sposto R, Finlay JL (2008). The Prognostic Value of Tumor Markers in Newly Diagnosed Patients with Primary Central Nervous System Germ Cell Tumors. Pediatric Blood & Cancer 51(6): 768-73.

› Kim L, Holland AJ, Srinivasan S, Cowell CT, Benn DE, Robinson BG (2008). Paediatric bilateral adrenal phaeochromocytomas in association with a novel mutation in the von Hippel Lindau gene. Journal of Paediatrics and Child Health 44(9): 514-6.

› Klassen TP, Hartling L, Craig JC, Offringa M (2008). Children are not just small adults: The urgent need for high-quality trial evidence in children. PLoS Medicine 5(8): e172.

› Kohnen S, Nickels L, Brunsdon R, Coltheart M (2008). Patterns of generalisation after treating sublexical spelling deficits in a child with mixed dysgraphia. Journal of Research in Reading 31(1), 157-177.

› Kohnen S, Nickels L, Coltheart M, Brunsdon R (2008). Predicting generalization in the training of irregular word spelling: Treating lexical spelling deficits in a child. Cognitive Neuropsychology 25 (3) 343-375.

› Kondo M, Gray LJ, Pelka GJ, Christodoulou J, Tam PP, Hannan AJ (2008). Environmental enrichment ameliorates a motor coordination deficit in a mouse model of Rett syndrome-Mecp2 gene dosage effects and BDNF expression. The European Journal of Neuroscience 27(12): 3342-50.

› Kozlowska K, Rose D, Khan R, Kram S, Lane L, Collins J (2008). A conceptual model and practice framework for managing chronic pain in children and adolescents. Harvard Review of Psychiatry 16(2): 136-50.

› Kwok SC, O’Loughlin EV, Kakakios AM, van Asperen PP (2008). Wheezy swallow: poorly responsive ‘asthma’. Journal of Paediatrics and Child Health 44(1-2): 74-7.

› Kylasam S, Mos K, Fijtin S, Webster B, Chard RB, Egan JR (2008). Recombinant activated Factor VII following pediatric cardiac surgery. Journal of Intensive Care Medicine 24(2):116-21.

› Lahra MM, Beeby PJ, Jeffery HE (2008). Maternal versus fetal inflammation and respiratory distress syndrome: a 10-year hospital cohort study. Archives of Disease in Childhood. Fetal and Neonatal Edition 94(1): F13-6.

› Lam LT (2008). Uncorrected or untreated vision problems and occupational injuries among the adolescent and adult population in Australia. Injury prevention: Journal of the International Society for Child and Adolescent Injury Prevention 14(6): 396-400.

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› Landorf KB, Burns J (2008). Health Outcome Assessment. Merriman’s Assessment of the Lower Limb. B. Yates. United Kingdom, Churchill Livingstone: 33-51.

› Lau L, Stuurman K, Weitzman S (2008). Skeletal Langerhan cell histiocytosis in children: Permanent consequences and health-related quality of life in long term survivors. Paediatric Blood and Cancer 50(3): 607-612.

› Lau LMS, Nugent JK, Zhao X, Irwin MS (2008). HDM2 antagonist Nutlin-3 disrupts p73-HDM2 binding and enhances p73 function. Oncogene 27: 997-1003.

› Lau YJ, Dao Q (2008). Cutaneous burns from a fiberoptic cable tip during arthroscopy of the knee. Knee 15(4): 333-5.

› Lawrence G, Gold MS, Hill R, Deeks S, Glasswell A, McIntyre PB (2008). Annual report: surveillance of adverse events following immunisation in Australia, 2007. Communicable Diseases Intelligence 32(4): 371-87.

› Leask J, Macartney K (2008). Parental decisions about vaccination: collective values are important. Journal of Paediatrics and Child Health 44(10): 534-5.

› Leask J, Quinn HE, Macartney K, Trent M, Massey P, Carr C, Turahui J (2008). Immunisation attitudes, knowledge and practices of health professionals in regional NSW. Australian and New Zealand Journal of Public Health 32(3): 224-9.

› Lee VWS, Wang YM, Wang YP, Zheng D, Polhill T, Cao Q, Wu H, Alexander IE, Alexander SI, Harris DC (2008). Regulatory immune cells in kidney disease. American Journal of Physiology. Renal Physiology 295(2): F335-42.

› Lee YC, Kelly DF, Yu LM, Slack MP, Booy R, Heath PT, Siegrist CA, Moxon RE, Pollard AJ (2008). Haemophilus influenzae type b vaccine failure in children is associated with inadequate production of high-quality antibody. Clinical Infectious Diseases: an Official Publication of the Infectious Diseases Society of America 46(2): 186-92.

› Leeflang MM, Deeks JJ, Gatsonis C, Bossuyt PM, Cochrane Diagnostic Test Accuracy Working Group (Irwig L, Craig JC – members) (2008). Systematic reviews of diagnostic test accuracy. Annals of Internal Medicine 149(12): 889-97.

› Lewis P, Wilson V (2008). Commentary on Booth J, Tolson D, Hotchkiss R, Schofield I (2007) Using action research to construct national evidence-based nursing care guidance for gerontological nursing. Journal of Clinical Nursing 16, 945-953, 17(12): 1671-3.

› Lewis SL, Khoo PL, De Young RA, Steiner K, Wilcock C, Mukhopadhyay M, Westphal H, Jamieson RV, Robb L, Tam PP (2008). Dkk1 and Wnt3 interact to control head morphogenesis in the mouse. Development (Cambridge, England) 135(10): 1791-801.

› Li M, Li S, Baur LA, Huxley RR (2008). A systematic review of school-based intervention studies for the prevention or reduction of excess weight among Chinese children and adolescents. Obesity Reviews: an Official Journal of the International Association for the Study of Obesity 9(6): 548-59.

› Little DG (2008). Humanizing a mouse gene for human therapeutics: lessons from denosumab. Journal of Bone and Mineral Research: The Official Journal of the American Society for Bone and Mineral Research 24(2): 179-81.

› Liu AJ, Briody JN, Munns CF, Waugh MCA (2008). Regional changes In Bone Mineral Density following Spinal Cord Injury in Children. Developmental Neurorehabilitation 11(1):51-59.

› Lo HP, Cooper ST, Evesson FJ, Seto JT, Chiotis M, Tay V, Compton AG, Cairns AG, Corbett A, MacArthur DG, Yang N, Reardon K, North KN (2008). Limb-girdle muscular dystrophy: diagnostic evaluation, frequency and clues to pathogenesis. Neuromuscular Disorders: NMD. 18(1): 34-44.

› Logan GJ, Wang L, Zheng M, Ginn SL, Coppel RL, Alexander IE (2008). Antigen-specific humoral tolerance or immune augmentation induced by intramuscular delivery of adeno-associated viruses encoding CTLA4-Ig-antigen fusion molecules. Gene Therapy 16(2): 200-10.

› London K, Howman-Giles R (2008). Ga-67 and CT fusion imaging of an infected aortic graft. Clinical Nuclear Medicine 33(1): 41-3.

› Loon SC, Liew G, Craig J, Fung A, Reid S (2008). Meta-analysis of randomised controlled trials comparing timolol with brimonidine in the treatment of glaucoma. Clinical and Experimental Ophthalmology 36(3): 281-9.

› Lord B, Ungerer J, Wastell C (2008). Implications of resolving the diagnosis of PKU for parents and children. Journal of Pediatric Psychology 33(8): 855-66.

› Ma CS, Chew GY, Simpson N, Priyadarshi A, Wong M, Grimbacher B, Fulcher DA, Tangye SG, Cook MC (2008). Deficiency of Th17 cells in hyper IgE syndrome due to mutations in STAT3. Journal of Experimental Medicine 205(7):1551-7.

› MacArthur DG, Seto JT, Chan S, Quinlan KG, Raftery JM, Turner N, Nicholson MD, Kee AJ, Hardeman EC, Gunning PW, Cooney GJ, Head SI, Yang N, North KN (2008). An Actn3 knockout mouse provides mechanistic insights into the association between alpha-actinin-3 deficiency and human athletic performance. Human Molecular Genetics 17(8): 1076-86.

› Macartney KK, Burgess MA (2008). Varicella vaccination in Australia and New Zealand. The Journal of Infectious Diseases 197 (2)S191-5.

› Machaalani R, Makris A, Thornton C, Hennessy A (2008). Vascular endothelial growth factor receptor 1 (Flt1) and apoptosis in the preeclamptic placenta and effects of in vivo anti-hypertensive exposure. Hypertens Pregnancy 27(4):361-73.

› Machaalani R, Say M, Waters KA (2008). Serotoninergic receptor 1A in the sudden infant death syndrome brainstem medulla and associations with clinical risk factors. Acta Neuropathologica [Epub].

› Machaalani R, Waters KA (2008). Neuronal cell death in the Sudden Infant Death Syndrome brainstem and associations with risk factors. Brain; A Journal of Neurology 131(Pt 1): 218-28.

› MacLean JE, Fitzsimons D, Hayward P, Waters K, Fitzgerald DA (2008). Screening for obstructive sleep apnoea in pre-school children with cleft palate. The Cleft Palate-Craniofacial Journal 46 (2) 117–123.

› MacLean JE, Fitzsimons D, Hayward P, Waters KA, Fitzgerald DA (2008). The identification of children with cleft palate and sleep disordered breathing using a referral system. Pediatric Pulmonology 43(3): 245-250.

› Magoffin A, Allen JR, McCauley J, Gruca MA, Peat J, Van Asperen P, Gaskin K(2008). Longitudinal analysis of resting energy expenditure in patients with cystic fibrosis. The Journal of Pediatrics 152(5): 703-8.

› Maguire AM, Biesheuvel CJ, Ambler GR, Moore B, McLean M, Cowell CT (2008). Evaluation of adrenal function using the human corticotrophin-releasing hormone test, low dose Synacthen test and 9am cortisol level in children and adolescents with central adrenal insufficiency. Clinical Endocrinology (Oxf) 68(5): 683-91.

› Maguire AM, Craig ME, Cowell CT (2008). Management of adrenal insufficiency during the stress of medical illness and surgery. Comment. The Medical Journal of Australia 189(6): 350.

› Marchant J, Cheng NG, Lam LT, Fahy FE, Soundappan SV, Cass DT, Browne GJ (2008). Bystander basic life support: an important link in the chain of survival for children suffering a drowning or near-drowning episode. The Medical Journal of Australia 188(8): 484-5.

› Margetts CDE, Morris M, Astuti D, Gentle DC, Cascon A, McRonald FE, Catchpoole DR, Robledo M, Neumann HPH, Latif F, Maher ER (2008). Evaluation of a functional epigenetic approach to identify promoter region methylation in phaeochromocytoma. Endocrine-Related Cancer 15, 777-786.

› Martin C, Gunning P (2008). Isoform sorting of tropomyosins. Advances in Experimental Medicine and Biology 644187-200.

› Martin PT, Shelton GD, Dickinson PJ, Sturges BK, Xu R, Lecouteur RA, Guo LT, Grahn RA, Lo HP, North KN, Malik R, Engvall E, Lyons LA (2008). Muscular dystrophy associated with alpha-dystroglycan deficiency in Sphynx and Devon Rex cats. Neuromuscular Disorders 18(12):942-52.

› McDonald MM, Dulai S, Godfrey C, Amanat N, Sztynda T, Little DG (2008). Bolus or weekly zoledronic acid administration does not delay endochondral fracture repair but weekly dosing enhances delays in hard callus remodeling. Bone 43(4): 653-62.

› McGrath KH, Caldwell PH, Jones MP (2008). The frequency of constipation in children with nocturnal enuresis: a comparison with parental reporting. Journal of Paediatrics and Child Health 44(1-2): 19-27.

› McIntyre P, Leask J (2008). Improving uptake of MMR vaccine. BMJ (editorial) 336(7647): 729-30.

› McKay K, Wilcken B (2008). Newborn screening for cystic fibrosis offers an advantage over symptomatic diagnosis for the long term benefit of patients: the motion for. Paediatric Respiratory Reviews 9(4): 290-4.

› Mehr SS, Kemp AS (2008). Feeding choice for children with immediate allergic reactions to cows milk protein. The Medical Journal of Australia 189(3): 178-9.

› Menzies R, Turnour C, Chiu C, McIntyre P (2008). Vaccine preventable diseases and vaccination coverage in Aboriginal and Torres Strait Islander people, Australia 2003 to 2006. Communicable Diseases Intelligence 32 Suppl: S2-67.

› Mihelec M, St Heaps L, Flaherty M, Billson F, Rudduck C, Tam PP, Grigg JR, Peters GB, Jamieson RV (2008). Chromosomal rearrangements and novel genes in disorders of eye development, cataract and glaucoma. Twin Research and Human Genetics: The Official Journal of the International Society for Twin Studies 11(4): 412-21.

› Mihrshahi S, Webb K, Almqvist C, Kemp AS, CAPS Team (2008). Adherence to allergy prevention recommendations in children with a family

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history of asthma. Pediatric Allergy and Immunology: Official Publication of the European Society of Pediatric Allergy and Immunology 19(4): 355-62.

› Moore P, Kerridge I, Gillis J, Jacobe S, Isaacs D (2008). Withdrawal and limitation of life-sustaining treatments in a paediatric intensive care unit and review of the literature. Journal of Paediatrics and Child Health 44(7-8): 404-8.

› Morgan PJ, Okely AD, Cliff DP, Jones RA, Baur LA (2008). Correlates of objectively measured physical activity in obese children. Obesity 16(12): 2634-41.

› Morrow AM, Quine S, Loughlin EV, Craig JC (2008). Different priorities: a comparison of parents’ and health professionals’ perceptions of quality of life in quadriplegic cerebral palsy. Archives of Disease in Childhood 93(2): 119-25.

› Moujaber T, MacIntyre CR, Backhouse J, Gidding H, Quinn H, Gilbert GL (2008). The seroepidemiology of Helicobacter pylori infection in Australia. International Journal of Infectious Diseases 12(5): 500-4.

› Nakamura K, Barzi F, Lam TH, Huxley R, Feigin VL, Ueshima H, Woo J, Gu D, Ohkubo T, Lawes CM, Suh I, Woodward M; Asia Pacific Cohort Studies Collaboration (2008). Cigarette smoking, systolic blood pressure, and cardiovascular diseases in the Asia-Pacific region. Stroke. A Journal of Cerebral Circulation 39(6): 1694-702.

› Nardone A, Tischer A, Andrews N, Backhouse J, Theeten H, Gatcheva N, Zarvou M, Kriz B, Pebody RG, Bartha K, O’Flanagan D, Cohen D, Duks A, Griskevicius A, Mossong J, Barbara C, Pistol A, Slaciková M, Prosenc K, Johansen K, Miller E (2008). Comparison of rubella seroepidemiology in 17 countries: progress towards international disease control targets. Bulletin of the World Health Organization 86(2):118-25.

› Nath CE, Earl JW, Pati N, Stephen K, Shaw PJ (2008). Variability in the pharmacokinetics of intravenous busulphan given as a single daily dose to paediatric blood or marrow transplant recipients. British Journal of Clinical Pharmacology 66(1): 50-9.

› Newall AT, Brotherton JM, Quinn HE, McIntyre PB, Backhouse J, Gilbert L, Esser MT, Erick J, Bryan J, Formica N, MacIntyre CR (2008). Population seroprevalence of human papillomavirus types 6, 11, 16, and 18 in men, women, and children in Australia. Clinical Infectious Diseases: An Official Publication of the Infectious Diseases Society of America 46(11): 1647-55.

› Newall AT, Scuffham PA (2008). Influenza-related disease: the cost to the Australian healthcare system. Vaccine 26(52): 6818-23.

› Newall AT, Scuffham PA, Kelly H, Harsley S, MacIntyre CR (2008). The cost-effectiveness of a universal influenza vaccination program for adults aged 50–64 years in Australia. Vaccine 26:2142-53.

› Newall AT, Wood JG, MacIntyre CR (2008). Influenza-related hospitalisation and death in Australians aged 50 years and older. Vaccine 26(17): 2135-41.

› North K (2008). What’s new in congenital myopathies? Neuromuscular Disorders: 18(6): 433-42.

› North K (2008). Why is alpha-actinin-3 deficiency so common in the general population? The evolution of athletic performance. Twin Research and Human Genetics: The Official Journal of the International Society for Twin Studies 11(4): 384-94.

› North KN, Laing NG (2008). Skeletal muscle alpha-actin disease. Advances in Experimental Medicine Biology 642:15027.

› Nunn GR, Bennetts J, Onikul E (2008). Durability of hand-sewn valves in the right ventricular outlet. The Journal of Thoracic and Cardiovascular Surgery 136(2): 290-6.

› O’Connor J, Steinbeck K, Hill A, Booth M, Kohn M, Shah S, Baur L (2008). Evaluation of a community-based weight management program for overweight and obese adolescents: The Loozit® study. Nutrition & Dietetics 65(2): 121-7.

› O’Neill GM, Stehn J, Gunning PW (2008). Tropomyosins as interpreters of the signalling environment to regulate the local cytoskeleton. Seminars in Cancer Biology 18(1): 35-44.

› O’Riordan SM, Robinson PD, Donaghue KC, Moran A, ISPAD Clinical Practice Consensus (2008). Management of cystic fibrosis-related diabetes. Pediatric Diabetes 9(4 Pt 1): 338-44.

› Othman N, Isaacs D, Daley AJ, Kesson AM (2008). Mycoplasma pneumoniae infection in a clinical setting. Pediatrics International: Official Journal of the Japan Pediatric Society 50(5): 662-6.

› Ouvrier R. (2008). Hyperventilation and the Pitt-Hopkins syndrome. Developmental Medicine and Child Neurology 50(7): 481.

› Pace RA, Peat RA, Baker NL, Zamurs L, Mörgelin M, Irving M, Adams NE, Bateman JF, Mowat D, Smith NJ, Lamont PJ, Moore SA, Mathews KD, North KN, Lamandé SR (2008). Collagen VI glycine mutations: perturbed assembly and a spectrum of clinical severity. Annals of Neurology 64(3): 294-303.

› Payton LA, Lewis JD, Byrne JA, Bright RK (2008). Vaccination with metastasis-related tumour associated antigen TPD52 and CpG/ODN induces protective tumour immunity. Cancer Immunology, Immunotherapy 57(6):799-811.

› Peadon E, Fremantle E, Bower C, Elliott EJ (2008). International survey of diagnostic services for children with Fetal Alcohol Spectrum Disorders. BMC Pediatrics [Epub] 812.

› Peadon EM, Bower CI, Elliott EJ (2008). Teenage smoking in pregnancy and birthweight: a population study, 2001-2004. The Medical Journal of Australia 189(4): 237-8.

› Pearce C, Leask J, Ritchie J (2008). Tapping midwives’ views about the neonatal hepatitis B vaccine: how welcome is a move towards a health promoting orientation? Health Promotion Journal of Australia: Official Journal of Australian Association of Health Promotion Professionals 19(2): 161-3.

› Peat RA, Gecz J, Fallon JR, Tarpey PS, Smith R, Futreal A, Stratton MR, Lamande SR, Yang N, North KN (2008). Exclusion of biglycan mutations in a cohort of patients with neuromuscular disorders. Neuromuscular Disorders: 18(8): 606-9.

› Peat RA, Smith JM, Compton AG, Baker NL, Pace RA, Burkin DJ, Kaufman SJ, Lamande SR, North KN (2008). Diagnosis and etiology of congenital muscular dystrophy. Neurology 71(5): 312-21.

› Perkovic V, Cass A, Patel AA, Suriyawongpaisal P, Barzi F, Chadban S, Macmahon S, Neal B; InterASIA Collaborative Group (2008). High prevalence of chronic kidney disease in Thailand. Kidney International 73(4): 473-9.

› Perkovic V, Verdon C, Ninomiya T, Barzi F, Cass A, Patel A, Jardine M, Gallagher M, Turnbull F, Chalmers J, Craig J, Huxley R (2008). The relationship between proteinuria and coronary risk: a systematic review and meta-analysis. PLoS Medicine 5(10): e207.

› Porter MA, Coltheart M, Langdon R (2008). Theory of mind in Williams syndrome assessed using a nonverbal task. Journal of Autism and Developmental Disorders 38, 806-814.

› Porter MA, Dodd H, Cairns D (2008). Psychopathological and behavior impairments in Williams-Beuren syndrome: The influence of gender, chronological age and cognition. Child Neuropsychology 15 (4), 359-374.

› Quak SH, Furnes R, Lavine J, Baur LA; Obesity Working Group (2008). Obesity in children and adolescents. Journal of Pediatric Gastroenterology and Nutrition 47(2): 254-9.

› Quijano-Roy S, Mbieleu B, Bönnemann CG, Jeannet PY, Colomer J, Clarke NF, Cuisset JM, Roper H, De Meirleir L, D’Amico A, Ben Yaou R, Nascimento A, Barois A, Demay L, Bertini E, Ferreiro A, Sewry CA, Romero NB, Ryan M, Muntoni F, Guicheney P, Richard P, Bonne G, Estournet B (2008). De novo LMNA mutations cause a new form of congenital muscular dystrophy. Annals of Neurology 64(2): 177-86.

› Rajan V, Bartlett N, Harvey JG, Martin HCO, La Hei ER, Arbuckle S, Godfrey C, Holland AJA (2008). Delayed Cooling of an Acute Scald Contact Burn Injury in a Porcine Model: is it Worthwhile? Journal of Burn Care Research 30(4):729-34.

› Rajan V, Holland AJA, Harvey JG (2008). The Impact of Laser Doppler Imaging on decision making in children with burns. ANZ Journal of Surgery 78(s. 1): A8.

› Rajan V, Murray RZ (2008). The duplicitous nature of inflammation in wound repair. Wound Practice & Research 16, 122-129.

› Rashid H, Booy R, Shafi S, Haworth E (2008). Conjugate versus polysaccharide meningococcal vaccine [letter]. Lancet Infectious Diseases 8(4):215.

› Rashid H, Haworth E, Shafi S, Memish ZA, Booy R (2008). Pandemic influenza: mass gatherings and mass infection [commentary]. Lancet Infectious Diseases 8:526-7.

› Rashid H, Shafi S, Booy R, El Bashir H, Ali K, Zambon MC, Memish ZA, Ellis J, Coen PG, Haworth E (2008). Influenza and respiratory syncytial virus infections in British Hajj pilgrims. Emerging Health Threats Journal 1:e2.

› Rashid H, Shafi S, Haworth E, Booy R, on behalf of the Health at Hajj and Umra Research Group (2008). Pneumococcal vaccination in adults [letter]. Archives of Internal Medicine 168:666-7.

› Rashid H, Shafi S, Haworth E, El Bashir H, Booy R (2008). Influenza and RSV among returning travellers. British Journal of Infection Control 9(4): 17-18.

› Rashid H, Shafi S, Haworth E, El Bashir H, Memish ZA, Sudhanva M, Smith M, Auburn H, Booy R (2008). Viral respiratory infections at the Hajj: comparison between UK and Saudi pilgrims. Clinical Microbiology and Infection 14(6):569-74.

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› Rashid H, Shafi S, Haworth E, Memish ZA, El Bashir H, Ali KA, Booy R. (2008). Influenza vaccine in Hajj pilgrims: policy issues from field studies. Vaccine 26(37): 4809-12.

› Rauch F, Plotkin H, DiMeglio L, Engelbert RH, Henderson RC, Munns C, Wenkert D, Zeitler P (2008). Fracture prediction and the definition of osteoporosis in children and adolescents: the ISCD 2007 Pediatric Official Positions. Journal of Clinical Densitometry 11(1):22-8.

› Ravindranathan H, Gillis J, Lord DJ (2008). Intensive care experience with sclerotherapy for cervicofacial lymphatic malformations. Pediatric Critical Care Medicine: a Journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies 9(3): 304-9.

› Rawlinson WD, Hall B, Jones CA, Jeffery HE, Arbuckle SM, Graf N, Howard J, Morris JM (2008). Viruses and other infections in stillbirth: what is the evidence and what should we be doing? Pathology 40(2): 149-60.

› Reddel S, Ouvrier RA, Nicholson G, Dierick I, Irobi J, Timmerman V, Ryan MM (2008). Autosomal dominant congenital spinal muscular atrophy--a possible developmental deficiency of motor neurones? Neuromuscular Disorders: NMD 18(7): 530-5.

› Redmond AC, Burns J, Ouvrier RA (2008). Factors that influence health-related quality of life in Australian adults with Charcot-Marie-Tooth disease. Neuromuscular Disorders: NMD 18(8): 619-25.

› Rhodes P, Madden S, Brown J, Baillie A (2008). Parent to Parent Consultation in the Maudsley Model of Family-Based Treatment for Anorexia Nervosa: A Randomised Control Trial. Journal of Family Therapy 30:96-108.

› Ridda I, Lindley IR, Gao Z, McIntyre P, Macintyre CR (2008). Differences in attitudes, beliefs and knowledge of hospital health care workers and community doctors to vaccination of older people. Vaccine 26(44): 5633-40.

› Ridda I, Lindley R, MacIntyre RC (2008). The challenges of clinical trials in the exclusion zone: the case of the frail elderly. Australasian Journal on Ageing 27(2): 61-6.

› Ridda I, Motbey C, Lam L, Lindley IR, McIntyre PB, Macintyre CR (2008). Factors associated with pneumococcal immunisation among hospitalised elderly persons: a survey of patient’s perception, attitude, and knowledge. Vaccine 26(2): 234-40.

› Robinson PD, Dalton D, Cripps T, Wood NJ, Kesson AM, Isaacs D (2008). Tuberculosis in children: a tertiary centre perspective. The Medical Journal of Australia 188(3): 190-1.

› Robinson PD, Waters K (2008). Are children just small adults? The differences between paediatric and adult sleep medicine. Internal Medicine Journal 38(9): 719-31.

› Rose KJ, Burns J, Ryan MM, Ouvrier RA, North KN (2008). Reliability of quantifying foot and ankle muscle strength in very young children. Muscle & Nerve 37(5): 626-31.

› Sandler G, Chennapragada SM, Soundappan SS, Cass D (2008). Pediatric high-flow priapism and super-selective angiography--an Australian perspective. Journal of Pediatric Surgery 43(10): 1898-901.

› Sandler G, Patrick E, Cass D (2008). Long standing balanitis xerotica obliterans resulting in renal impairment in a child. Pediatric Surgery International 24(8): 961-4.

› Sandler G, Soundappan SS, Cass D (2008). Appendicitis and low-flow priapism in children. Journal of Pediatric Surgery 43(11): 2091-5.

› Scheinberg A, O’Flaherty S, Waugh M, Baxter A, Gurd K (2008). Letters to the editor. Journal of Paediatrics and Child Health 44(7-8): 468-9.

› Schevzov G, Fath T, Vrhovski B, Vlahovich N, Rajan S, Hook J, Joya JE, Lemckert F, Puttur F, Lin JJ, Hardeman EC, Wieczorek DF, O’Neill GM, Gunning PW (2008). Divergent regulation of the sarcomere and the cytoskeleton. The Journal of Biological Chemistry 283(1): 275-83.

› Schindeler A, Little DG (2008). Recent insights into bone development, homeostasis, and repair in type 1 neurofibromatosis (NF1). Bone 42(4): 616-22.

› Schindeler A, McDonald MM, Bokko P, Little DG (2008). Bone remodeling during fracture repair: The cellular picture. Seminars in Cell & Developmental Biology 19(5): 459-66.

› Schindeler A, Morse A, Harry L, Godfrey C, Mikulec K, McDonald M, Gasser JA, Little DG (2008). Models of tibial fracture healing in normal and Nf1-deficient mice. Journal of Orthopaedic Research: Official Publication of the Orthopaedic Research Society 26(8): 1053-60.

› Schindeler A, Ramachandran M, Godfrey C, Morse A, McDonald M, Mikulec K, Little DG (2008). Modeling bone morphogenetic protein and bisphosphonate combination therapy in wild-type and Nf1 haploinsufficient mice. Journal of Orthopaedic Research: Official Publication of the Orthopaedic Research Society 26(1): 65-74.

› Schmalzl L, Palermo R, Coltheart M (2008). Cognitive heterogeneity in genetically-based prosopagnosia: A family study. Journal of Neuropsychology 2, 99-117.

› Schmalzl L, Palermo R, Green M, Brunsdon R, Coltheart M (2008). Training of familiar face recognition and visual scan paths for faces in a child with congenital prosopagnosia. Cognitive Neuropsychology 25 (5), 704-72.

› Schünemann HJ, Oxman AD, Brozek J, Glasziou P, Jaeschke R, Vist GE, Williams Jr JW, Craig J, Montori VM, Bossuyt P, Guyatt GH (2008). GRADE: grading quality of evidence and strength of recommendations for diagnostic tests and strategies. BMJ 336: 1106-10.

› Seale H, Dwyer DE, MacIntyre CR, Chapman J (2008). Cytomegalovirus disease amongst renal transplant recipients in Australia and New Zealand. Virology: Research and Treatment 1: 65-73.

› Seale H, MacIntyre CR, Dwyer DE (2008). The epidemiology of cytomegalovirus disease in HIV-infected patients before and after the introduction of highly active antiretroviral therapy. The Open Epidemiology Journal 1:57-61.

› Sengupta N, Booy R, Schmitt HJ, Peltola H, Van-Damme P, Schumacher RF, Campins M, Rodrigo C, Heikkinen T, Seward J, Jumaan A, Finn A, Olcén P, Thiry N, Weil-Olivier C, Breuer J (2008). Varicella vaccination in Europe: are we ready for a universal childhood program? European Journal of Pediatrics 167(1): 47-55.

› Shafi S, Booy R, Haworth E, Rashid H, Memish ZA (2008). Hajj: Health lessons for mass gatherings. Journal of Infection and Public Health 1: 27-32.

› Shehata M, Bieche I, Boutros R, Weidenhofer J, Fanayan S, Spalding L, Zeps N, Byth K, Bright RK, Lidereau R, Byrne JA (2008). Nonredundant functions for tumor protein D52-like proteins support specific targeting of TPD52. Clinical Cancer Research: An Official Journal of the American Association for Cancer Research 14(16): 5050-60.

› Shehata M, Weidenhofer J, Thamotharampillai K, Hardy JR, Byrne JA (2008). Tumor protein D52 overexpression and gene amplification in cancers from a mosaic of microarrays. Critical Reviews in Oncogenesis 14(1): 33-55.

› Sheikh M, MacIntyre CR, Perera S (2008). Preventive detention: the ethical ground where politics and health meet. Focus on asylum seekers in Australia. Journal of Epidemiology and Community Health 62(6): 480-3.

› Singh J, Saxena A, Christodoulou J, Ravine D (2008). MECP2 genomic structure and function: insights from ENCODE. Nucleic Acids Research 36(19): 6035-47.

› Sinn JKH, Badawi N (2008). Cerebral Palsy in the Young Infant: A Diagnostic Challenge. Paediatrics and Child Health in General Practice 3:4-5.

› Skinner R, Brotherton J (2008). Research highlights: highlights from the latest articles in human papillomavirus vaccination and cervical cancer research. Therapy 5:283-7.

› Smith LJ, van Asperen PP, McKay KO, Selvadurai H, Fitzgerald DA (2008). Reduced exercise capacity in children born very preterm. Pediatrics 122(2): e287-93.

› Smith MA, Canfell K, Brotherton JM, Lew JB, Barnabas RV (2008). The predicted impact of vaccination on human papillomavirus infections in Australia. International Journal of Cancer. Journal International du Cancer 123(8): 1854-63.

› Soh NL, Touyz S, Dobbins T, Surgenor L, Clarke S, Kohn M, Lee EL, Leow V, Rieger E, Ung KE, Walter G (2008). Cross-cultural differences in the macronutrient intakes of women with anorexia nervosa in Australia and Singapore. European Eating Disorders Review: The Journal of the Eating Disorders Association 16(6): 427-35.

› Somerville H, Tzannes G, Wood J, Shun A, Hill C, Arrowsmith F, Slater A, O’Loughlin EV (2008). Gastrointestinal and nutritional problems in severe developmental disability. Developmental Medicine and Child Neurology 50(9): 712-6.

› Soo C, Tate R, Williams L, Waddingham S, Waugh MC (2008). Development and validation of the Paediatric Care and Needs Scale for children and youth with acquired brain injury. Developmental Neurorehabilitation 11:3 204-214.

› Soundappan SV, Martin H, Cass DT (2008). Unusual neck sinus--first or second cleft? Journal of Pediatric Surgery 43(7): e5-7.

› Spelman D, Buttery J, Daley A, Isaacs D, Jennens I, Kakakios A, Lawrence R, Roberts S, Torda A, Watson DA, Woolley I, Anderson T, Street A, Australasian Society for Infectious Diseases (2008). Guidelines for the prevention of sepsis in asplenic and hyposplenic patients. Internal Medicine Journal 38(5): 349-56.

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› Spilchak PJ, Denney-Wilson E, King L, Baur LA (2008). Tertiary paediatric obesity services in Australia. Journal of Paediatrics and Child Health 44(5): 243-7.

› Steinbeck K, Baur L, Cowell C, Pietrobelli A (2008). Clinical research in adolescents: challenges and opportunities using obesity as a model. International Journal of Obesity 33(1): 2-7.

› Steinbeck KS, Brodie L, Towns SJ (2008). Transition in chronic illness: Who is going where? Journal of Paediatrics and Child Health 44(9): 478-82.

› Stone ML, Walker JL, Chisholm D, Craig ME, Donaghue KC, Crock P, Anderson D, Verge CF (2008). The addition of rosiglitazone to insulin in adolescents with type 1 diabetes and poor glycaemic control: a randomized-controlled trial. Pediatric Diabetes 9(4 Pt 1): 326-34.

› Strippoli GFM, Navaneethan S, Johnson DM, Perkovic V, Pellegrini F, Nicolucci A, Craig JC (2008). Effects of statins in patients with chronic kidney disease: meta-analysis and meta-regression of randomised controlled trials BMJ 336(7645): 645-51.

› Strowig T, Brilot F, Bougras G, Muller WA, Münz C (2008). Tonsilar Natural killer cells restrict B cell transformation by the Epstein Barr virus. PLoS Pathogens 4(2): e27.

› Strowig T, Brilot F, Münz C (2008). Non-cytoxic functions of natural killer cells: direct pathogen restriction and assistance to adaptative immunity. Journal of Immunology 180: 7785-7791.

› Sugiana C, Pagliarini DJ, McKenzie M, Kirby DM, Salemi R, Abu-Amero KK, Dahl HH, Hutchison WM, Vascotto KA, Smith SM, Newbold RF, Christodoulou J, Calvo S, Mootha VK, Ryan MT, Thorburn DR (2008). Mutation of C20orf7 disrupts complex I assembly and causes lethal neonatal mitochondrial disease. American Journal of Human Genetics 83(4): 468-78.

› Swinburn B, Sacks G, Lobstein T, Rigby N, Baur LA, Brownell KD, Gill T, Seidell J, Kumanyika S, International Obesity Taskforce Working Group on Marketing to Children (2008). The ‘Sydney Principles’ for reducing the commercial promotion of foods and beverages to children. Public Health Nutrition 11(9): 881-6.

› Takawira F, Ayer JG, Onikul E, Hawker RE, Kemp A, Nicholson IA, Sholler GF (2008). Evaluation of the extracardiac conduit modification of the Fontan operation for thrombus formation using magnetic resonance imaging. Heart, Lung & Circulation 17(5): 407-10.

› Tang S, Machaalani R, Waters KA (2008). Brain-derived neurotrophic factor (BDNF) and TrkB in the piglet brainstem after post-natal nicotine and intermittent hypercapnic hypoxia. Brain Research 1232: 195-205.

› Thomas P, Joseph TL, Menzies RI (2008). Evaluation of a targeted immunisation program for Aboriginal and Torres Strait Islander infants in an urban setting. NSW Public Health Bulletin 19:96-9.

› Thoms JA, Loch HM, Bamburg JR, Gunning PW, Weinberger RP (2008). A tropomyosin 1 induced defect in cytokinesis can be rescued by elevated expression of cofilin. Cell Motility and the Cytoskeleton 65(12): 979-90.

› Thoo CH, Graf N, Hogan P (2008). Erythema induratum in a Kenyan child. The Australasian Journal of Dermatology 49(3): 156-8.

› Timperio A, Salmon J, Ball K, Baur LA, Telford A, Jackson M, Salmon L, Crawford D (2008). Family physical activity and sedentary environments and weight change in children. International Journal of Pediatric Obesity: IJPO: An Official Journal of the International Association for the Study of Obesity 3(3): 160-7.

› Tomkins KL, Holland AJ (2008). Electrical burn injuries in children. Journal of Paediatrics and Child Health [Epub].

› Tong A, Chapman S, Sainsbury P, Craig JC (2008). An analysis of media coverage on the prevention and early detection of CKD in Australia. American Journal of Kidney Diseases: The Official Journal of the National Kidney Foundation 52(1): 159-70.

› Tong A, Lowe A, Sainsbury P, Craig JC (2008). Experiences of parents who have children with chronic kidney disease: a systematic review of qualitative studies. Pediatrics 121(2): 349-60.

› Tong A, Sainsbury P, Carter SM, Hall B, Harris DC, Walker RG, Hawley CM, Chadban S, Craig JC (2008). Patients’ priorities for health research: focus group study of patients with chronic kidney disease. Nephrology, Dialysis, Transplantation: Official Publication of the European Dialysis and Transplant Association - European Renal Association 23(10): 3206-14.

› Tong A, Sainsbury P, Craig JC (2008). Support interventions for caregivers of people with chronic kidney disease: a systematic review. Nephrology, Dialysis, Transplantation: Official Publication of the European Dialysis and Transplant Association - European Renal Association 23(12): 3960-5.

› Troedson C, Gill D, Dale RC (2008). Emergence of acute necrotising encephalopathy in Australia. Journal of Paediatrics and Child Health 44(10): 599-601.

› Tsang TW, Kohn M, Chow CM, Singh MF (2008). Health benefits of Kung Fu: a systematic review. Journal of Sports Sciences 26(12): 1249-67.

› Turnbull F, Neal B, Ninomiya T, Algert C, Arima H, Barzi F, Bulpitt C, Chalmers J, Fagard R, Gleason A, Heritier S, Li N, Perkovic V, Woodward M, MacMahon S, Blood Pressure Lowering Treatment Trialists’ Collaboration (2008). Effects of different regimens to lower blood pressure on major cardiovascular events in older and younger adults: meta-analysis of randomised trials. BMJ (Clinical Research ed.) 336(7653): 1121-3.

› Turnbull F, Woodward M, Neal B, Barzi F, Ninomiya T, Chalmers J, Perkovic V, Li N, MacMahon S; Blood Pressure Lowering Treatment Trialists’ Collaboration (2008). Do men and women respond differently to blood pressure-lowering treatment? Results of prospectively designed overviews of randomized trials. European Heart Journal 29(21): 2669-80.

› Viner RM, Clark C, Taylor SJ, Bhui K, Klineberg E, Head J, Booy R, Stansfeld SA (2008). Longitudinal risk factors for persistent fatigue in adolescents. Archives of Pediatric and Adolescent Medicine 162(5):469-75.

› Vlahovich N, Schevzov G, Nair-Shaliker V, Ilkovski B, Artap ST, Joya JE, Kee AJ, North KN, Gunning PW, Hardeman EC (2008). Tropomyosin 4 Defines Novel Filaments in Skeletal Muscle Associated With Muscle Remodelling/Regeneration in Normal and Diseased Muscle. Cell Motility and the Cytoskeleton 65(1):73-85.

› Walker K, Badawi N, Hamid CH, Vora A, Halliday R, Taylor C, Shi E, Roy GT, Simpson E, Holland AJ (2008). A population-based study of the outcome after small bowel atresia/stenosis in New South Wales and the Australian Capital Territory, Australia, 1992-2003. Journal of Pediatric Surgery 43(3): 484-8.

› Wallen M, Ziviani J, Herbert R, Evans R, Novak I (2008). Modified constraint-induced therapy for children with hemiplegic cerebral palsy: a feasibility study. Developmental Neurorehabilitation 11(2): 124-33.

› Walton J, Gibson WP, Sanli H, Prelog K (2008). Predicting cochlear implant outcomes in children with auditory neuropathy. Otology & Neurotology: Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology 29(3): 302-9.

› Wang H, Deeks S, Glasswell A, McIntyre P (2008). Trends in invasive Haemophilus influenzae type B disease in Australia, 1995-2005. Communicable Diseases Intelligence 32(3): 316-25.

› Wang YM, Hu M, Wang Y, Polhill T, Zhang GY, Wang Y, Lee VW, Harris DC, Alexander SI (2008). Regulatory T cells in renal disease. International Journal of Clinical and Experimental Medicine 1(4): 294-304.

› Ward KA, McIntyre PB, Kirkwood CD, Roche PW, Ferson MJ, Van Buynder PG, Roberts-Witteveen AR, Kesson AM, Krause VL, McAnulty JM (2008). Rotavirus surveillance in Australia. Communicable Diseases Intelligence 32(1): 82-7.

› Waters K (2008). Interventions in the paediatric sleep laboratory: the use and titration of respiratory support therapies. Paediatric Respiratory Reviews 9(3): 181-91.

› Watkins RE, Cooke FC, Donovan RJ, MacIntyre CR, Itzwerth R, Plant AJ (2008). Tackle the problem when it gets here: pandemic preparedness among small and medium businesses. Qualitative Health Research 18(7):902-12.

› Watt SE, Shores A, North KN (2008). An examination of lexical and sublexical reading skills in children with neurofibromatosis type 1. Child Neuropsychology: A Journal on Normal and Abnormal Development in Childhood and Adolescence 14(5): 401-18.

› Webster AC, Wong G, Craig JC, Chapman JR (2008). Managing cancer risk and decision making after kidney transplantation. American Journal of Transplantation 8(11): 2185-91.

› Webster RI, Erdos C, Evans K, Majnemer A, Saigal G, Kehayia E, Thordardottir E, Evans A, Shevell MI (2008). Neurological and magnetic resonance imaging findings in children with developmental language impairment. Journal of Child Neurology 23(8): 870-7.

› Wegener C, Burns J, Penkala S. (2008). Effect of neutral-cushioned running shoes on plantar pressure loading and comfort in athletes with cavus feet: a crossover randomized controlled trial. The American Journal of Sports Medicine 36(11): 2139-46.

› Wells CA, Salvage-Jones JA, Li X, Hitchens K, Butcher S, Murray RZ, Beckhouse AG, Lo YS, Manzanero S, Cobbold C, Schroder K, Ma B, Orr S, Stewart L, Lebus D, Sobieszczuk P, Hume DA, Stow J, Blanchard H, Ashman RB (2008). The Macrophage-Inducible C-Type Lectin, Mincle, Is an Essential Component of the Innate Immune Response to Candida albicans. Journal of Immunology 180: 7404 - 7413.

› Whatham A, Bartlett H, Eperjesi F, Blumenthal C, Allen J, Suttle C, Gaskin K (2008). Vitamin and mineral deficiencies in the developed world and their effect on the eye and vision. Ophthalmic & Physiological Optics:

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Publications

Publications

The Journal of the British College of Ophthalmic Opticians (Optometrists) 28(1): 1-12.

› Wiebe PN, Blimkie CJ, Farpour-Lambert N, Briody J, Marsh D, Kemp A, Cowell C, Howman-Giles R (2008). Effects of single-leg drop-landing exercise from different heights on skeletal adaptations in prepubertal girls: a randomized controlled study. Pediatric Exercise Science 20(2):211-228.

› Wilcken B (2008). More on medium-chain acyl-coenzyme a dehydrogenase deficiency in a neonate. The New England Journal of Medicine 358(6): 647.

› Wilcken B (2008). The consequences of extended newborn screening programs: Do we know who needs treatment? Journal of Inherited Metabolic Disease [Epub].

› Wilcken B, Wiley V (2008). Newborn screening. Pathology 40(2): 104-15.

› Williams G, Fletcher JT, Alexander SI, Craig JC (2008). Vesicoureteral reflux. Journal of the American Society of Nephrology 19(5): 847-62.

› Williams K, Helmer M, Duncan GW, Peat JK, Mellis CM (2008). Perinatal and maternal risk factors for autism spectrum disorders in New South Wales, Australia. Child: Care, Health and Development 34(2): 249-56.

› Williams LM, Hermens DF, Palmer D, Kohn M, Clarke S, Keage H, Clark CR, Gordon E (2008). Misinterpreting emotional expressions in attention-deficit/hyperactivity disorder: evidence for a neural marker and stimulant effects. Biological Psychiatry 63(10): 917-26.

› Wilson M, Peters G, Bennetts B, McGillivray G, Wu ZH, Poon C, Algar E (2008). The clinical phenotype of mosaicism for genome-wide paternal uniparental disomy: two new reports. American Journal of Medical Genetics. Part A 146A(2): 137-48.

› Wilson V, McCormack B, Ives G (2008). Developing healthcare practice through action learning: Individual and group journeys. Action Learning: Research and Practice 5(1), 21-38.

› Wiltshire EJ, Mohsin F, Chan A, Donaghue KC (2008). Methylenetetrahydrofolate reductase and methionine synthase reductase gene polymorphisms and protection from microvascular complications in adolescents with type 1 diabetes. Pediatric Diabetes 9(4 Pt 2): 348-53.

› Wong E, Kumar V, Howman-Giles RB, Vanderheyden JL (2008). Imaging of Therapy-Induced Apoptosis Using (99m)Tc-HYNIC-Annexin V in Thymoma Tumor-Bearing Mice. Cancer Biotherapy & Radiopharmaceuticals [Epub].

› Wong G, Chapman JR (2008). Cancers after renal transplantation. Transplantation Reviews (Orlando, Fla.) 22(2): 141-9.

› Wong G, Chapman JR, Craig JC (2008). Cancer screening in renal transplant recipients: what is the evidence? Clinical Journal of the American Society of Nephrology 3 (2) S87-100.

› Wong G, Howard K, Chapman JR, Craig JC (2008). Cost-effectiveness of breast cancer screening in women on dialysis. American Journal of Kidney Diseases: The Official Journal of the National Kidney Foundation 52(5): 916-29.

› Wong G, Howard K, Craig JC, Chapman JR (2008). Cost-effectiveness of colorectal cancer screening in renal transplant recipients. Transplantation 85(4): 532-41.

› Wong L, Hunt A, Burns J, Crosbie J (2008). Effect of foot morphology on center-of-pressure excursion during barefoot walking. Journal of the American Podiatric Medical Association 98(2): 112-7.

› Wood J, MacIntyre R, Macartney K (2008). Assessing varicella vaccine efficacy [letter]. Pediatric Infectious Disease Journal 27(2):190.

› Wood N, McIntyre P (2008). Pertussis: review of epidemiology, diagnosis, management and prevention. Paediatric Respiratory Reviews 9(3): 201-11.

› Wood N, Quinn HE, McIntyre P, Elliott E (2008). Pertussis in infants: preventing deaths and hospitalisations in the very young. Journal of Paediatrics and Child Health 44(4): 161-5.

› Wood N, Warlow M, Quinn H, Selvey C, Lum G, McIntyre P, Kaldor J (2008). Establishment of a surveillance system (utilising Midwifes Data Collection Systems) for monitoring the impact of hepatitis B vaccination on the population prevalence of chronic hepatitis B virus infection in Australia. Australian and New Zealand Journal of Public Health 32(3): 272-5.

› Yapo BR, Gerges B, Holland AJ (2008). Investigation and management of suspected urachal anomalies in children. Pediatric Surgery International 24(5): 589-92.

› Yee A, De Ravin SS, Elliott E, Ziegler JB; Contributors to the Australian Paediatric Surveillance Unit (2008). Severe combined immunodeficiency: a national surveillance study. Pediatric Allergy and Immunology: Official Publication of the European Society of Pediatric Allergy and Immunology 19(4): 298-302.

› Young DJ, Bebbington A, Anderson A, Ravine D, Ellaway C, Kulkarni A, de Klerk N, Kaufmann WE, Leonard H (2008). The diagnosis of autism in a female: could it be Rett syndrome? European Journal of Pediatrics 167(6): 661-9.

› Young HK, Barton BA, Waisbren S, Portales Dale L, Ryan MM, Webster RI, North KN (2008). Cognitive and psychological profile of males with Becker muscular dystrophy. Journal of Child Neurology 23(2): 155-62.

› Yu NYC, Ruys AJ, Zenios M, Godfrey C, McDonald M, Kiely P, Mikulec K, Little DG, Schindeler A (2008). Bisphosphonate-laden acrylic bone cement: mechanical properties, elution performance, and in vivo activity. Journal of Biomedical Materials Research. Part B, Applied Biomaterials 87(2): 482-91.

› Yuan FF, Marks K, Wong M, Watson S, de Leon E, McIntyre PB, Sullivan JS (2008). Clinical relevance of TLR2, TLR4, CD14 and FcgammaRIIA gene polymorphisms in Streptococcus pneumoniae infection. Immunology and Cell Biology 86(3): 268-70.

› Zeng L, Nath CE, Shaw PJ, Earl JW, McLachlan AJ (2008). HPLC-fluorescence assay for acyclovir in children. Biomedical Chromatography 22(8): 879-87.

› Zhang GY, Hu M, Wang YM, Alexander SI (2008). Foxp3 as a marker of tolerance induction versus rejection. Current Opinion in Transplantation 14(1):40-5.

› Zhu K, Greenfield H, Du X, Zhang Q, Ma G, Hu X, Cowell C, Fraser DR (2008). Effects of two years’ milk supplementation on size-corrected bone mineral density of Chinese girls. Asia Pacifc Journal of Clinical Nutrition 17 (1):147-150.

› Zhu K, Greenfield H, Zhang Q, Du X, Ma G, Foo LH, Cowell CT, Fraser DR (2008). Growth and bone mineral accretion during puberty in Chinese girls: a five-year longitudinal study. Journal of Bone and Mineral Research: The Official Journal of the American Society for Bone and Mineral Research 23(2): 167-72.

› Zurynski Y, Elliott EJ (2008). Australian Paediatric Surveillance Unit annual report, 2006. Communicable Diseases Intelligence 32(1): 52-6.

› Zurynski YA, Bilston L, Elliott EJ (2008). Booster seat use by children aged 4-11 years: evidence of the need to revise current Australasian standards to accommodate overweight children. The Medical Journal of Australia 189(3): 183.

› Zurynski YA, Frith K, Leonard H, Elliott E (2008). Rare childhood diseases: how should we respond? Archives of Disease in Childhood 93(12): 1071-4.

› Zurynski YA, Lester-Smith D, Festa MS, Kesson AM, Booy R, Elliott EJ (2008). Enhanced surveillance for serious complications of influenza in children: role of the Australian Paediatric Surveillance Unit. Communicable Diseases Intelligence 32(1): 71-6.

› Zurynski YA, Mahajan D, Elliott EJ (2008). Australian Paediatric Surveillance Unit annual report 2007. Communicable Diseases Intelligence 32(4): 430-4.

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Our supporters

Our supporters

thank youThe research conducted at the Kids Research Institute at The Children’s Hospital at Westmead is heavily reliant on the additional financial support we receive from the community. Through your generosity our teams of researchers are able to tackle some of the most complex problems in paediatric medicine and, in many cases, transform the knowledge they acquire into tangible benefits for patients.

This report has highlighted some of the many advances and achievements our researchers have made in the past year alone. There are many children and young people who are alive today thanks to the research carried out here, and many more who look to our future

Benefactors and DonorsWe are fortunate to have received more than $7 million in donations towards research conducted across all of our research streams. Our supporters have a joint commitment to ensuring that the sickest children receive not just the best compassionate care from dedicated teams at The Children’s Hospital at Westmead, but also that those children and their families have available to them the best possible research to support their treatment.

The Kids Research Institute is incredibly grateful for the generous support of the following individuals and organisations.

advancements with great hope. None of this would be possible without your help.

But it does not stop there. We have some of the most talented and dedicated medical research staff in the world at the Kids Research Institute. They need access to the most up-to-date tools and resources and support for their research programs and clinical trials.

We need your sustained support to ensure we have enough recurring funds this year and in years to come to maintain the momentum. It is only with your help that we will be able to continue to deliver the best possible outcomes for sick children across the country.

BenefactorEstate Late Elizabeth Lottie M Rosenthal

DonorsEstate Late Clifton Albert EllisMrs Alice StylesEstate Late Archibald Rawson BurchDr Rachel SkinnerFuture of FishBeverley CotteeEstate Late Clarence James DaleyMrs BG GowingMr Michael HockeyMr & Mrs Halil & Hatice Ibrahim & UyanikMs Marion MaxwellMrs R McNeillMr Daniel MongEstate Late Cornelius Gerardus Mulders The Children’s Hospital at Westmead - Neuroinflammatory Research GroupThe Children’s Hospital at Westmead - Orthoptics

AchievementsThe contributions of our supporters have helped to enhance the Kerry Packer facility which houses the Kids Research Institute. Since construction of the facility in 2005 we have been able to grow our research capacity by 30%, ensuring that more research is being conducted to protect the future of our children. Your support has made that possible.

In the next year our plans centre on:

- Building our clinical trials capacity

- Continuing and growing our research programs

- Ensuring we have the right equipment and facilities available for our researchers

- Providing internal grants to our research units and renowned researchers to allow new, innovative research to take place

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How you can help

How you can help

Your gift today will play a vital role in enabling us to understand, treat and cure childhood diseases, as well as improving the quality of life for children suffering from serious illness for which there is no cure.

Research has uncovered the causes of many diseases, yet aggressive persistence is required for more cures and treatments to be developed. Those children who have managed to win their battle against life-threatening illnesses would not be here today without the critical breakthroughs that medical research has achieved.

Clinical trials, tests and research cost a staggering amount of money. Without sufficient funding, the cure

for many childhood diseases will never be found. That is why your support is so important to ensure that our work continues - so that our children have the best possible hope of a future.

Every gift makes a difference. By lending your support to one of our Research Streams or to our Research Infrastructure or Endowment programs, you will be supporting the quest for cures for diseases, where as yet none have been found.

For more details of the priority funding requirements of the Kids Research Institute, please contact our Fundraising Department on 02 9845 3367.

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PublicationsPublications

How you can help

How you can help

To send a donation via cheque or mail, please direct your donation to: Research Office Kids Research Institute at The Children’s Hospital at Westmead Locked Bag 4001 Westmead NSW 2145

(Cheques should be made payable to The Children’s Hospital at Westmead).

To make a donation by phone, please call our Fundraising Department on 02 9845 3367.To donate online please visit www.kidsresearch.org.au

Kate Geraghty/FAIRFAXPHOTOS.COM.

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The Children’s Hospital at Westmead

Locked Bag 4001

Westmead NSW 2145

Tel: (02) 9845 3367

Web: www.chw.edu.au