undertaking rare disease research and surveillance dr chris verity, consultant paediatric...

Undertaking rare disease research and surveillance

Dr Chris Verity, Consultant Paediatric Neurologist

#raredisease

Whooping cough

vaccination

Pertussis notifications and vaccine uptake 1940 - 2005

0

50,000

100,000

150,000

200,00019

40

1945

1950

1955

1960

1965

1970

1975

1980

1985

1990

1995

2000

2005

years

noti

fica

tion

s of

pe

rtus

sis

0

20

40

60

80

100

vacc

ine

cove

rage

notifications coverage

Pertussis immunisation and serious acute neurologicalillness in children

D L Miller, E M Ross, R Alderslade, M H Bellman, N S B Rawson BMJ 1981, 282, 1595-9

• 1000 cases notified to the National Childhood Encephalopathy Study between 1976 and 1979. Diagnoses included encephalitis/encephalopathy, prolonged convulsions, infantile spasms, and Reye syndrome.

• Conclusion: pertussis vaccine not the cause of “brain damage”.

1986

The British Paediatric Surveillance Unit

The epidemiology of progressive intellectual and neurological deterioration in childhood

Verity CM, Winstone AM, Stellitano L, Will R, Nicoll A

Arch Dis Child 2010;95:361-4

The PIND StudyA spectrum of

neurodegenerative diseases

Searching for a needle in a haystack

Progressive Intellectual and Neurological Deterioration (PIND)

Case definition

Child 16 years or less at onset with:• progressive deterioration > 3months• loss of intellectual/developmental abilities• abnormal neurological signs

Even if diagnosis already made

PIND Study – 3445 reported casesMay 1997 – July 2013

No case, 1450

Under investigat-ion, 231

No diagnosis,

168

vCJD, 6

Definite diagnosis,

1475

Out-standing,

115

Prion protein genotypes in the population

VV, 12%

MV, 51%

MM, 37%

All confirmed vCJD cases: methionine homozygotes (MM)

Methionine/valine genotypes might have longer incubation

vCJD has not gone away

1976

National influenza immunisation programme suspended following reports of Guillain-Barre

syndrome following swine ’flu vaccination

December 2009

British Paediatric Surveillance Unit Report Card

HIV & AIDS

Progressive Intellectual & Neurological Deterioration

Congenital Rubella

Severe Neonatal Hypernatraemia

Guillain-Barré syndrome / Fisher syndrome (UK Only)

CNS Inflammatory Demyelinating Disease

Gonorrhoea, Syphilis, Chlamydia, and Trichomonas infectious

Congenital Syphilis

March 2010 [1003]

CODE No [ ]Nothing to Report Specify in box the number of cases seen

ConclusionsDH England

Children: H1N1 vaccn recorded in 855,378

Our study (whole of UK): 112 GBS/3 FS cases

93/115 cases had preceding infections

2: H1N1 vaccination < 3m before GBS

3: seasonal ‘flu vaccination > 3m before

Outcome better than in studies of adults

Support the BPSU – cut down bureacracy

BPSU surveillance as seen from space