pediatrecs emq

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Pediatrics 1

Pediatrics

1) Theme: ABDOMINAL PAIN

A. PeritonitisB. AppendicitisC. ConstipationD. Urinary tract infectionE. Mesenteric lymphadenitis

F. MigraineG. IntussusceptionH. Henoch-Schonlein vasculitisI. PsychosomaticJ. Shigella dysentry

For the following patients with abdominal pain, choose the most likely diagnosis.

1) A previously well 10 month old baby presenting withdrawing up of her legs with crying and some bloody runnystools.

G. Intussusception

Note: This is the right age group for intussception. It is often preceded by an URTI and it is thought thatmesenteric lymphadenopathy may precipitate intussception. A red currant jelly-like stool is a late sign. Inthe older child, the cause of intussception may be HSP or lymphoma.

2) A 4-year-old child, with poor eating habits, iron deficiencyanaemia and intermittent colicky abdominal pain.

C. Constipation

Note: Constipation is common in children and should be managed aggressively to establish regular bowel habitquickly.

3) A 2-year-old girl with nephrotic syndrome who hasdeveloped a fever and abdominal pain.

A. Peritonitis

Note: The causes of abdominal pain in nephrotic syndrome are peritonitis (usually pneumococcal), renal veinthrombosis and hypovolaemia.

2) Theme: Skin lesions

A. Café au lait spotsB. Capillary haemangiomaC. Port-wine stainD. Shagreen patchE. Adenoma sebaceum

F. Cutis MarmorataG. Herald's patchH. Peri-oral pigmentationI. Hypo pigmentationJ. Tuber

Select one option from the list above that is most suitable for the following patients

1) An 11 year old boy with learning difficulties has an acne typerash over the nose

E. Adenoma sebaceum

Note: relates to the condition known as Tuberous sclerosis. It is a condition where by hamartomatous lesionsbecome evident in early childhood. Seizures and learning difficulties are common and skin manifestationsinclude fibrous angiomatous lesions in the naso labial folds known as adenoma sebaceum. White maculesare seen over the trunk known as Ash leaf macules and Shagreen patches resemble goose like flesh.Subungual fibromata are also common.

2) A 4 year old boy with a history of focal seizures and a birthmark

C. Port-wine stain

Note: relates to Sturge-Weber syndrome. The association and localization of aberrant vasculature in the facialskin, eyes and meninges. Most commonly in the trigeminal nerve distribution.

3) An infant with Down's syndrome has mottled appearance tothe skin.

F. Cutis Marmorata

Note: relates to Cutis Marmorata, a mottled, marbled type appearance to the skin. Common in Down'ssyndrome as well as other Trisomies, hypothyroidism and Cornelia-de-Lange syndrome.


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3) Theme: Poisoning

A. Ant psychoticsB. BenzodiazepinesC. Ethylene glycolD. MethanolE. Opiates

F. OrganophosphatesG. SalicylatesH. SympathomimeticI. Tricyclic antidepressantsJ. Volatile solvents

Select the most appropriate agent from the above list that would elicit the following toxic effects:

1) Coma, pinpoint pupils, hyperventilation E. Opiates

2) Hyper salivation, broncho-rrhoea, broncho- spasms,perspiration, neuromuscular paralysis

F. Organophosphates

3) Tachypnoea, metabolic acidosis, Haematemesis, ReyesSyndrome.

G. Salicylates

Comments: A pinpoint pupil suggests : opiates (Heroin – Morphine – codeine). The hyper salivation, tachypnoeaand sweating suggest :Organophosphate poisoning. This drug inhibits ant-cholinesterase activitypromoting Ach effects. Treatment consists of anticholinergics < (Atropine dose i.v 0.05 mg/kg repeatedevery 5-10 min as needed adverse effect tachycardia, dry mouth, blurred vision, and urinary retention).Finally, the use of salicylates in children is well-recognized cause of Reyes syndrome, a conditionassociated with fulminant hepatic failure. Suspected poisoning in children result in thousands ofattendances at A&E department each year. Various drugs cause specific signs when taken in overdose.Careful examination of the child should follow A, B, C protocol.

4) Theme: Chemotherapy

Select the most appropriate chemotherapeutic agent from the above list that corresponds withthe following actions and side effects:

1) A drug disrupting microtubule leading to loss of deep tendon reflexes. J. Vincristine

2) A drug inhibiting initiation of DNA synthesis resulting in conjunctivitisand cerebellar toxicity.

G. Cytosine arabinoside

3) A drug resulting in dose related lung damage. D. Bleomycin

Comments:

All cytotoxic agents have adverse effects. General side-effects include nausea,vomiting and bone marrow suppression, alopecia and stomatitis. More specific side-

effects are listed in the questions above. Alkylating agents such as Cyclophosphamide

may cause a haemorrhagic cystitis. Asparaginase results in a dose related Pancreatitis.

The anthracyclines e.g. Doxorubicin and Daunorubicin are cardiotoxic, which is often

very difficult to detect. Monitoring with echocardiograms is advised. Vincristine is an

alkaloid agent and results in sensory motor neuropathy with long-term use. It also has

an affect on the autonomic system resulting in severe constipation and paralytic ileus.

It may also result in sensory changes with parathesis progressing to loss of tendon

reflexes. Bleomycin is the main drug resulting in lung damage and occurs in up to 10%

of patients. The damage is dose related.


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Pediatrics 3

5) Theme: Childhood malignancies

A. Burkitt's nasopharyngeal carcinomaB. Ewing sarcomaC. HepatoblastomaD. Hodgkin's lymphomaE. Melanoma

F. Nesidio-blastosisG. NeuroblastomaH. TeratomaI. Wilm's tumourJ. Xeroderma pigmentosum

Select the most appropriate oncological diagnosis from the above list of options to explain thefollowing scenarios

1) A 13-year-old boy presents with abdominal pain. An abdominal massis found. This boy has a past medical history of biliary atresia.

C. Hepatoblastoma

2) A malignant transformation of a skin resulting from an autosomalrecessive condition.

J. Xeroderma pigmentosum

3) A baby on the neonatal unit is found to be hypoglycaemic. F. Nesidio-blastosis

Comments: Hepatoblastoma is a malignant liver tumour. It may present with anorexia, abdominal mass and jaundice.It is associated with cirrhosis as well as biliary cirrhosis secondary to biliary atresia. Xerodermapigmentosa is an autosomal recessive condition of defective DNA repair. Skin damage arise andmalignant transformation results at multiple sites. Nesidio-blastosis is also known as beta cell endocrinebenign hyperplasia and affects the pancreas. Diagnosis is made by hypoglycaemia with high serumInsulin and treatment is by Pancreatectomy.

6) Theme: SYNDROMES

A. Down's syndromeB. Turner's syndromeC. Noonan's syndrome

D. Klinefelter's syndromeE. Treacher-Collins syndrome

F. Marfan's syndromeG. Sotos syndromeH. Klippel-feil syndrome

I. Laurence Moon Biedl syndromeJ. MCune Albright syndrome

Which is the most applicable diagnosis for the following children.

1) A 3-year-old boy with delayed motor development is reviewed regularly in the child development clinic.He attends for routine annual blood tests to check his thyroid function. A. Down's syndrome

Note: Children with Down’s syndrome are screened yearly for the development of hypothyroidism. They shouldalso be regularly assessed for the development of myopia and hearing loss. These children have apredisposition to acute lymphoblastic leukaemia (ALL), Alzheimer’s-like dementia and Atlanto-axialsubluxation.

2) A 16-year-old girl is being investigated for delayed menarche and short stature. In her notes it isrecorded that she had swollen feet at birth but she was lost to follow-up. B. Turner's syndrome

Note: A karyotype should be performed in all girls presenting in this way. Pedal lymphoedema at birth is aknown presentation. Other associated features include coarctation of the aorta, infertility, hypothyroidismand horse shoe kidney.

3) A girl of 6 years has been found to have hypertension and coarctation of the aorta. She has beenreferred to the growth clinic because of short stature. B. Turner's syndrome

Note: A karyotype should be performed in all girls presenting in this way. Pedal lymphoedema at birth is aknown presentation. Other associated features include coarctation of the aorta, infertility, hypothyroidism

and horse shoe kidney.


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7) Theme: PRECOCIOUS PUBERTY

A. CraniopharyngiomaB. Exaggerated adrenarcheC. McCune Albright syndromeD. Hypothalamic hamartoma

E. Thelarche variant

F. Neurofibromatosis type 1G. Granulosa-theca cell tumourH. HypothyroidismI. Premature thelarche

J. Congenital adrenal hyperplasia

Which is the most likely diagnosis in the following cases:

1) A 5-year-old girl presents with rapid breast development, breast stage 4, over a few months and anabdominal mass. There was no pubic hair or cliteromegaly.

Note: G. Granulosa-theca cell tumour The rapid onset of puberty is indicative of a tumour. In this case the alpha-fetoprotein tumour marker willbe raisedG. Symptoms result from the very high oestradiol levels.

2) An 8 year old boy presents with testicular volumes of 8 mls bilaterally, pubic hair stage 3 and genitalstage 3. He has long-standing constipation, deteriorating school performance and increased weight.

Note: H. Hypothyroidism

Long standing hypothyroidism can cause precocious puberty through high levels of TRH drivinggonadotrophin secretion and cross reactivity of the FSH receptor with TRH.

3) A 2year old girl presents with isolated bilateral breast development. Examination is otherwise normalas is her height velocity. Subsequent investigation demonstrates undetectable oestradiol levels and aprepubertal response to an LHRH test.

Note: I. Premature thelarche There may be a slight FSH predominance on the basal gonadotrophin levels. Premature thelarche is self-limiting and thought to be the result of the body being exquisitely sensitive to low levels of oestradiol.

8) Theme: Drugs During Pregnancy

A. Amino glycosidesB. AspirinC. Beta BlockersD. LithiumE. Phenytoin

F. PethidineG. Sodium ValproateH. SulphonamidesI. Thiazide diureticsJ. Warfarin

Select the drug from the above list of options that is most likely to be associated with thefollowing effects if taken during pregnancy:

1) This drug when taken in late pregnancy may result in the baby developing jaundiceH. Sulphonamides

2) This drug when taken in pregnancy may result in premature closure of the ductus arteriosis.B. Aspirin

3) Prolonged use of this drug may result in a permanent hearing impairment.A. Aminoglycosides

Comments:Sulphonamides taken in late gestation may compete with Bilirubin for protein binding

sites resulting in a neonatal hyperbilirubinaemia.

Aspirin has been associated with premature closure of the ductus arteriosis as well as

haemorrhagic disorders.

Aminoglycosides (such as Gentamicin) infused over a prolonged period during

pregnancy will result in ototoxicity in approximately 2-3 % of cases.


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Pediatrics 6

The description is of Guillain-Barre syndrome, which may be preceded by an URTI. There are lowermotor neurone signs but also with absent reflexes due to progressive distal demyelination. Sometimesthere is cranial nerve involvement (Miller Fisher syndrome)

3) A 9-year-old boy has a gradual onset of proximal weakness in the pelvic girdle and to a lesser extentin the shoulder girdle also. There is a faint violaceous rash over the eyelids and some telangiecasia over

the finger knuckles. He has a low-grade fever and the thigh and buttock muscles are notably tender onexamination.

F. Dermatomyositis

Note: Proximal myopathy, heliotrope rash over eyelids and the description of Gotrens papules over theknuckles indicate dermatomyositis.

11) Theme: DELAYED MILESTONES

A. 4 weeksB. 2 monthsC. 7 monthsD. 10 months

E. 12 months

F. 18 monthsG. 2 yearsH. 3 yearsI. 5 years

J. 6 years

At what age would you refer a child for investigation if they were unable to do the following:-

1) Sit unsupportedD. 10 months

2) Walk independentlyF. 18 months

3) SmileB. 2 months

Comments:

It is important to be familiar with the developmental milestones, particularly up to the age of 3 years.Milestones are grouped under 4 headings:

• Gross motor

• Fine motor

• Hearing and language

• Social

You should know the age range for each milestone. Developmental milestones are in Illingworth: 'TheNormal Child'.

12) Theme: Renal disease

A. Acute nephritisB. Nephrotic syndromeC. Diabetes insipidusD. Urinary tract infectionE. Chronic renal failure

F. Diabetes mellitusG. Salt poisoningH. Haemolytic uraemic syndromeI. Barrter syndromeJ. Cystinosis

Which diagnosis is most appropriate for the following patients?

1) A 2-year-old girl has become puffy in her face and has developed swollen feet. Her urine dipstick

shows proteinuria. B. Nephrotic syndrome

Note:


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Hypoalbuminaemia (serum albumin falls below 25g/L); oedema, proteinuria (exceed 2g/24 hr. or morethan 40 mg/m2/hr.)(In urine dipstick +3 – +4 for proteinuria) and hypercholestrolaemia (LDL G,triglyceridesG) are features of nephrotic syndrome. So are peritonitis (pneumococcal peritonitis due to lowimmunoglobulins), renal vein thrombosis and hypotension (due to hypovolaemia & hypercoagulable statewith low antthrombin III) .

2) A 15 years old boy has felt tired, lost his appetite and has not grown in the last year. His serum urea is20mmol/l and serum creatinine is 575Omol/l. His Hb is 9.6g/dl.

E. Chronic renal failure

Note: Chronic renal failure may have an insidious onset and may present with slowed poor growth and delayedpuberty.

3) A 2 week old boy presents to casualty with weight loss and inconsolable crying. His serum sodium is150mmol/l and his urine osmolality is 100 mosm/kg (normal > 600).

C. Diabetes insipidus

Note: Hypernatraemia with concomitant low urine osmolality suggest DI. Remember it is important to establishwhether nephrogenic in origin (treatment indomethacin or chlorthiazide – thiazides – carbamzepine -

chloramphenicol) or cranial (treatment DDAVP = exogenous ADH = Desmopressin = ADH analogue).

13) Theme : Neonatal Jaundice

A. ABO incompatibilityB. Breast milk jaundiceC. CephalhaematomaD. Congenital biliary atresiaE. Congenital cytomegalovirus infectionF. Congenital haemolytic anaemiaG. Congenital spherocytosis

H. GalactosaemiaI. Glucose-6-phosphate dehydrogenase deficiencyJ. Haemolytic disease of the newbornK. HypothyroidismL. Preterm birthM. Sepsis

A 6 day old infant is admitted with jaundice (serum bilirubin 300 micromol/l) and feeding poorly

for the past 48 hours. The infant was born at a gestational age of 36 weeks and weighed 2680g atbirth. The following are some of the clinical findings and results of investigations. In each case,select the most likely cause of the jaundice from the list of options.

1) The infant was born after a difficult forceps delivery and the head appeared to be swollen in the rightparieto-occipital area. Jaundice developed after 24 hours. The haemoglobin is 12 g/dl; there is indirecthyperbilirubinaemia.

C. Cephalhaematoma

2) The baby has a temperature of 38oC and tends to bring up its feeds. The circulating white cell count

is 18x109 /l. Microscopy of urine from a suprapubic aspirate shows large numbers of neutrophilpolymorphs.

M. Sepsis

3) The liver and spleen are palpable and there is a family history of lethargy, hepatosplenomegaly andpoor feeding. The raised bilirubin is initially unconjugated; in the second sample, the conjugated fractiontended to rise. Urine examination is positive for reducing substances using Clinitest.

H. Galactosaemia

4) On initial assessment, petechiae and hepatosplenomegaly are noted, and on investigation,thrombocytopenia and partially conjugated hyperbilirubinaemia are found. Chorioretinitis is noted laterand intra-cranial calcification is reported on a CT brain scan.

E. Congenital cytomegalovirus infection

5) The baby was mostly breast fed and 20% of the bilirubin is conjugated. Formula milk feeds caused arapid fall in bilirubin levels.

B. Breast milk jaundice


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Comments: 1. A cephalhaematoma is a subperiosteal swelling which can arise as a result of forceps delivery.Complications result from anaemia due to bleeding into the haematoma, and jaundice due to haemolysiswithin it2. Sepsis likely due to urinary tract infection.3. Galactosaemia is an autosomal recessive condition due to the absence of galactose-1-phosphateuridyl transferase. This results in the intracellular accumulation of galactose-1-phosphate which is toxic.

Upon commencement of milk feeds the child develops jaundice, vomiting, diarrhoea and failure to thrive.4. Congenital Cytomegalovirus infection is typically characterized by the signs described. CNS damagefrom congenital CMV infection exceeds that due to any other infectious cause.5. Breast milk jaundice is defined as : neonatal jaundice persisting for more than 28 days, with a totalbilirubin level greater than 5.9mg/dl, in an otherwise healthy, thriving, breast-fed infant.

14) Theme : Connective tissue disease

A. Ankylosing spondylitisB. DermatomyositisC. Ehlers Danlos syndromeD. Henoch Schonlein purpuraE. Juvenile chronic arthritis

F. Kawasaki diseaseG. Osteogenesis imperfectaH. Rheumatic feverI. SclerodermaJ. Systemic lupus erythematosus

Select one of the above diagnoses that would explain the following presentations:

1) A 10 year old boy presents with arthralgia, an urticarial rash on the lower limbs and buttocks andabdominal pain.

D. Henoch Schonlein purpura

Note: Henoch Schonlein purpura also known as anaphylactoid purpura result in non-thrombocytopenic =(normal plat. Count) purpura nodules, arthritis, abdominal pain and nephritis.

2) A six year old girl presents with fever, arthritis and a photosensitive rash. Dipstick of her urine revealsproteinuria.

J. Systemic lupus erythematosus

Note:

describes a child with SLE. A multi system disorder which results in fever, malaise, arthritis, rashestypically photosensitive rash and a butterfly rash. Kidneys are also affected resulting inglomerulonephritis. Diagnosis is made by elevated S anti-nuclear antibody, Double stranded DNA withmuscle antibodies.

3) A 4 year old boy is admitted to casualty with fracture of her ulna after minor injury. She is noted tohave blue sclerae and hearing impairment.

G. Osteogenesis imperfecta

Note: Osteogenesis imperfecta, a syndrome of fragile bones due to defect in the T-chain of type 1collagen,which can be divided in to 4 separate types. There is widespread osteopaenia resulting in bone fragility,bow legs. Individuals characteristically have blue sclerae and hearing impairment (conductive deafness).The condition has variable inheritance with autosomal dominant and recessive forms, the latter beingmore severe.

Blue sclera – differential diagnosis1) Marfan syndrome2) Ehler-Danlos syndrome3) Osteogenesis imperficta4) Pseudo-xanthoma elasticum

15) Theme : NEPHROLOGY

A. Acute nephritisB. Nephrotic syndromeC. Diabetes insipidusD. Urinary tract infectionE. Chronic renal failure

F. Diabetes mellitusG. Salt poisoningH. Haemolytic uraemic syndromeI. Barrter syndromeJ. Cystinosis

Which diagnosis is most appropriate for the following patients?


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17) Theme : Emergency treatment

A. 100% Oxygen plus nebulised SalbutamolB. 100% oxygen, adrenaline and hydrocortisoneC. Adrenaline IVD. DexamethasoneE. Hyperbaric oxygen

F. IntubationG. IV Hydrocortisone plus oral anti-HistaminesH. Paralysis with Sodium ThiopentoneI. Skin desensitisationJ. Supportive treatment

Select the most appropriate emergency treatment for the following cases

1) A 13 year old boy is brought to A&E with a grossly swollen face and difficulty breathing following abee sting. YZ[\ Y]^_

B. 100% oxygen, adrenaline and hydrocortisone

2) A 2 year old boy presents with a barking cough and stridor.D. Dexamethasone

3) A 4 month old baby with cough fever and mild respiratory distress has widespread crackles onauscultation.

J. Supportive treatment

Comments: 1) describes an anaphylactic reaction also known as angioneurotic oedema. In this case there is difficultybreathing i.e. life-threatening. Treatment includes 100% oxygen, IM adrenaline and IV Hydrocortisone.Intubation may be necessary.2) The second case has croup as characterized by the barking cough. Humidified oxygen can help formild cases but more severe cases require dexamethasone.3) The principles of management for bronchiolitis includes supportive treatment only. There is no specifictreatment other than humidified oxygen; broncho dilator therapy may be of some use.

18) Theme : Fine Motor Development

A. 3 months

B. 6 monthsC. 9 monthsD. 12 monthsE. 18 months

F. 2 years

G. 3 yearsH. 4 yearsI. 5 yearsJ. 6 years

For each description of a child's fine motor development choose, from the list of options, thehighest development age the child is most likely to have achieved.

1) Can copy a circle and a cross and climbs and descends stairs, one foot per step.H. 4 years

2) A child can scribble and draw a straight line but not yet a circle.F. 2 years

3) Spontaneously scribbles Y`abW with a crayon UcWdae and is starting to build a tower.E. 18 months

Comments:This question relates to the fine motor area of development.1) At 4 months the infant is able to grasp big objects and move them in to the mid-line.2) At 6 months the baby can grasp an object such a rattle and starts to transfer it from hand tohand. He or she also starts to discover the rest of his or her body parts.3) The pincer grip develops between 6 or 9 months and by 10 months the fine pincer grip isachieved with the baby being able to pick up small pellet like objects between thumb andforefinger.

4) At 18 months the baby starts to spontaneously scribble if offered a crayon for example5) At 24 months the child is able to copy a straight line.6) By age 3 years the child can copy a circle.

http://www.nlm.nih.gov/medlineplus/ency/article/000959.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000959.htm


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7) By 4 years can copy cross.8) By 4½ years can copy a square.9) By 5 years of age can copy a triangle10) A diamond at about 6 years of age.11) At the age of 3 the child starts to draw people usually starting as a “head and stick”extremity person and as development progresses the person becomes more sophisticated.

19) Theme : Causes of vomiting

A. Cyclical vomitingB. Duodenal atresiaC. Over feedingD. Meckel's diverticulumE. Ulcerative colitis

F. Meconium ileusG. Gastro-enteritisH. Gastro-oesophageal refluxI. Pyloric stenosisJ. Urinary tract infection


1) A 2 day old breast fed baby is vomiting after each feed. Abdominal x-ray demonstrates a double

bubble.B. Duodenal atresia

Note: Duodenal atresia is the most common cause of duodenal obstruction in infancy. It is frequently found inbabies with Down's syndrome. Babies present with bilious or non-bilious vomiting and a “double bubble”are seen on abdominal x-ray. This is the result of distension of the stomach and duodenum with aconstricting pylorus between them.

2) A one year old boy with a history of intermittent bleeding pr presents with features of an interceptionD. Meckel's diverticulum

Note: Meckel's diverticulum is most common under the age of 2. The Meckel's diverticulum is remnant of the

vitilo-intestinal duct and can present with features of intussusception or diverticulitis. Commonly presentswith painless bleeding and is diagnosed by a Meckel's scan.

3) A 4 month old baby who is thriving has persistent vomiting which is occasionally blood stained.H. Gastro-oesophageal reflux

Note: Gastro-oesophageal reflux. Reflux of gastric contents in to the oesophagus is a common event in infancy.Once the abdominal segment of the oesophagus lengthens in the first few months of life reflux improves.

20) Theme : Drugs During Pregnancy

A. AminoglycosidesB. AspirinC. Beta BlockersD. LithiumE. Phenytoin

F. PethidineG. Sodium ValproateH. SulphonamidesI. Thiazide diureticsJ. Warfarin

Select the drug from the above list of options that is most likely to be associated with thefollowing effects if taken during pregnancy:

1) This drug taken in early pregnancy may result in Ebstein's anomaly in 3% of cases.D. Lithium

2) This drug may result in a neural tube defect in approximately 2% of cases.G. Sodium Valproate

3) This drug, when taken in pregnancy may result in a baby being born with cranio-facial abnormalities,


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growth impairment and learning difficulties.E. Phenytoin

Comments:1) Lithium, a drug used in the treatment of bipolar affective disorder. If given in early

pregnancy is associated with a wide variety of cardiac defects with approximately 8%

having severe cardiac disease, Ebstein's anomaly being the most common (3% of all

cases).

2) Sodium Valproate tends to induce a neural tube defect (spina bifida) in

approximately 2% of pregnancies and therefore should be avoided. It is also associated

with abnormalities of the orofacial and digits, compromising the foetal Valproate

syndrome.

3) Phenytoin is well known for causing the foetal hydantoin syndrome, compromising

cranio-facial abnormalities and learning difficulties as well growth impairment.

21) Theme : Abdominal Pain

A. Acute gastro-enteritisB. AppendicitisC. ConstipationD. Diabetes MellitusE. Henoch Schonlein Purpura

F. Mesenteric AdenitisG. NephroblastomaH. PancreatitisI. Sickle Cell crisisJ. Wilm's Tumour


1) A 12 year old boy with a 3 week history of coryza is brought to A&E complaining of severe abdominalpain. He has swollen ankles and nodules on his buttocks, which do not disappear with pressure.

E. Henoch Schonlein Purpura

Note: Henoch Schonlein purpura also known as anaphylactoid purpura has an unknown aetiology. Childrenpresent with abdominal pain, possibly melaena due to haemorrhage and oedema of the gut wall. Theymay have a flitting arthritis affecting large joints. The nephritis presents with microscopic haematuria andproteinuria and the macular, papular, purple rash is rather characteristic with buttocks and extensivesurfaces of the legs and arms being affected.

2) A 10 year old girl has just returned from Thailand. She is brought to history of severe diarrhoeaassociated with abdominal pain. Her mother, a known diabetic, has similar symptoms.

A. Acute gastro-enteritis

Note: relates to a child with gastroenteritis. In the developed world gastroenteritis is very common and usually

mild. 60% of cases are due to viruses for example Rotavirus. In bacterial gastroenteritis; fever is commonand children present with colicky abdominal pain, vomiting, diarrhoea and possibly dehydration.

3) A 12 year old girl with a 2 month history of weight loss and increased appetite weight loss is brought toCasualty with severe abdominal pain. She denies any history of vomiting or excessive exercise. Hermother says that the child has been wetting her bed over the past 4 months.

D. Diabetes Mellitus

Note: describes a child with diabetes mellitus. The commonest metabolic / endocrine problem in childhood.Diabetes mellitus results from low Insulin levels resulting in abnormal metabolism of carbohydrate,protein and fats. Children present with polyuria, polydipsia and polyphagia. Children sometimes complainof abdominal discomfort and there is a history of weight loss.


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22) Theme : Causes of Headaches

A. Benign intra-cranial hypertensionB. Cerebral tumourC. Cluster headacheD. EncephalitisE. Extradural haematoma

F. MeningitisG. MigraineH. Subdural haemorrhageI. Sub arachnoid haemorrhageJ. Tension headache

Select the most appropriate diagnosis from the above list that would explain the following cases.

1) A 16 year old girl develops headache and vomiting. She has a fever and develops a non-blanchingrash.

F. Meningitis

2) A 17 year old male with a 4 day history of cough and fever presents with altered sensorium andvomiting.

D. Encephalitis

3) A 10 year old girl with a 6 month history of episodic abdominal pain presents with a severe headache,a complete ophthalmoplegia which lasts several days.

G. Migraine

Comments:1) Bacillus Meningitis is a severe infection that carried serious morbidity and mortality. It may presentsuddenly or insidiously. Most commonly symptoms start off as being non-specific, for example fever,anorexia, irritability and later develop in to a rash and shock. More specific signs include neck stiffnessand seizures.2) Encephalitis refers to inflammation of the brain and children present with an encephalopathy. Initialsymptoms may be non-specific or flu-like followed by changes in mental state, seizures andcomplications include cerebral oedema, cardiovascular instability, fluid and electrolyte disturbances.Herpes viruses have been implicated as well as other viruses and often there is a devastatingconsequence.3) Migraine is a recurrent headache with symptom free interval. Other symptoms include abdominal pain,

nausea or vomiting, aura, visual sensory or motor (photophobia may also occur). Other visualdisturbances may take the form of blurred vision, fortification spectra and flashing lights. Complicatedmigraine occurs when there are neurological signs during a headache that may persist after theheadache has terminated. For example hemiplegic migraine.

23) Theme : Dyspnoea in children

A. Acute severe asthmaB. Cardiac failureC. CostochondritisD. Diabetic ketoacidosisE. Hysterical over-breathing

F. Inhalation of foreign bodyG. Pleura effusionH. Pulmonary embolismI. Tension pneumothoraxJ. Pleural effusion

Select the most appropriate diagnosis from the options above that best explains the followingcases:

1) A 3 month old baby presents with tachycardia and tachypnoea with widespread crepitationsB. Cardiac failure

2) A 3 year old presents with cough and haemoptysis. Examination reveals reduced breath sounds overone lung and a localized wheeze.

F. Inhalation of foreign body

3) A boy presents with a dry cough, lethargy and shortness of breath. Examination reveals reducedbreath sounds over one side which is dull to percussion.

J. Pleural effusion


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Comments:Heart failure – A number of congenital heart lesions will result in cardiac failure, commonly in the firstyear of life. The baby becomes breathless particularly after crying or feeding. The baby may be havingdifficulty completing feeds and as a result there may be a problem with poor weight gain. Babies also areseen to have sweating as a prominent symptom. On examination the baby may be tachycardic andtachypnoenic. A murmur may be present or a gallop rhythm. Oedema may be seen around thedependant parts of the body. Pulmonary crepitations may also be heard. It may be possible to pick up

heart enlargement clinically and chest x-ray may reveal cardiomegaly and plethoric lung fields. In thesecond case the unilateral nature of the reduced breath sounds and wheeze strongly suggestinspissation of a foreign body. The final case has again unilateral signs but on this occasion the dullnessto percussion suggest a pleural effusion.

24) Theme : Abdominal diseases of Childhood

A. AerophagiaB. Alpha 1 antitrypsin deficiencyC. GalactosaemiaD. Hirschsprung's diseaseE. Intussusception

F. Lactose intoleranceG. Mesenteric adenitisH. Reyes syndromeI. ThalassaemiaJ. Wilson's disease

Select one of the above options that is the most appropriate diagnosis for the following cases:1) Usually presents with chronic liver disease often in association with neurological features.

J. Wilson's disease

Note: describes a child with Wilson's disease also known as hepatolenticular degeneration. It is an autosomalrecessive condition characterized by cirrhosis of the liver, degenerative changes of the brain and Kayser-Fleischer rings on the cornea. It is as a result of low Caeruloplasmin and low Copper excretion.

2) A 9 month old baby presents with a sudden onset of abdominal pain and loose stools which have theappearance of red currant jelly.

E. Intussusception

Note: Intussusception is an invagination of the bowel in to an adjacent segment. It presents as paroxysmal pain

occasionally with vomiting and palpation may reveal a sausage shaped mass : in the right upperabdomen. A delay in diagnosis results in the child passing blood stained mucus rectally some timesknown as red currant jelly. Intussusception can sometimes be reduced hydro-statically, if unsuccessfulsurgery is required.

3) A 4 day old infant presents with severe abdominal distension and feculent vomitingD. Hirschsprung's disease

Note: Hirschsprung's disease is absence of ganglia cells in part or all of the wall of the colon. The childpresents with bilious or feculent vomiting, constipation and distension.

25) Theme : Gross motor development

A. 1 monthB. 2 monthsC. 4 monthsD. 6 monthsE. 8 months

F. 10 monthsG. 1 yearH. 18 monthsI. 2 yearsJ. 3 years

For each description of a child's gross motor development choose, from the list of options, thehighest development age the child is most likely to have achieved.

1) A baby can crawl, weight bare and is starting to cruise.F. 10 months

2) A baby rolls from front to back and back to front however is not yet sitting unsupported.

D. 6 months


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3) A child ascends stairs using alternating feet and is able to stand on one foot for a few seconds.J. 3 years

Comments: This question relates to gross motor skills area of development.

1. At 3 months when placed prone an infant can lift his head with his arms extended.2. By 4 months head lag is lost and this is evident when raised from supine to the sitting position.3. At 5 months the baby starts to roll over and4. At 6 months the baby is starting to sit up with support for example cushions.5. Sitting unsupported is usually seen by 6-7 months6. At 9 months the baby can start to crawl.7. Cruising is usually achieved by about 10 months.8. At 12 months the baby can raise ijVk independently and often starts to take a few steps alone.9. At 18 months the toddler can run although this is usually in a tiff fashion. He or she can ascend

stairs with assistance10. At 20 months the child can climb the stairs holding on to a rail.11. At 3 years the child can ascend stairs in an adult fashion using alternating feet per step however

is unable to descend the stairs in such a fashion until 4 years of age.

26) Theme : Treatment of Diarrhoeal illness

A. MetronidazoleB. Gluten free dietC. No actionD. SteroidsE. Lactose free diet

F. Pancreatic enzyme supplementsG. Vitamin DH. High fibre dietI. Breast milk allergyJ. Praziquantel


1) A 2 year old girl is taken to the A&E department by her mother who says that her child has diarrhoeaand describes the stool as looking like peas and carrots.

C. No action

Note: Describes a child with toddler diarrhoea due to a fast gut transit time. Children between the ages of 6months and 3 years may have this complaint which manifests as a chronic diarrhoea. Children areotherwise healthy and thriving with all investigations carried out being normal.

2) A 7 year old child presents with a 3 month history of abdominal pain. She is found to have anaemiaand colonoscopy reveals a cobble stone appearance.

D. Steroids

Note: Relates to inflammatory bowel disease, Crohn's disease and ulcerative colitis respectively. In Crohn'sdisease the colonoscopy will reveal :cobbled stone mucosal appearances with fistulae where as inulcerative colitis : (crypt abscesses + Collar button ulcers)are present.

3) A 12 year old girl whose height and weight are on the 3rd centile complains of diarrhoea with a historythat the stools are difficult to flush away. She has an itchy rash over her limbs.

B. Gluten free diet

Note: relates to Coeliac disease. A sensitivity and intolerance to the gliadin protein : in rice, wheat and barley.Children present with failure to thrive, diarrhoea, abdominal pain, anorexia and abdominal distension.Diagnosis is suspected by elevated (anti-gliadin / anti-reticulin / anti-endomysial) antibodies andconfirmed by jejunal biopsies.


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27) Theme : TREATMENT OF CHILDHOOD CONDITIONS

A. AcyclovirB. CefotaximeC. ErythromycinD. GentamicinE. Immunoglobulin

F. No medicationG. ParacetomolH. PenicillinI. PrednisoloneJ. Quinine

All of the following children present with a febrile illness. Based on the symptoms presentedbelow, select the single treatment option most likely to alter the clinical course of the disease ineach case. Each option may be used once, more than once, or not at all

1) A playful and active 5 year old boy presents with a low grade fever and bruising. The rest of theexamination is normal. His platelet count is 14 x 109 /L

F. No medication

Note: Childhood thrombocytopenia may occur in the setting of viral illness.

2) A 10 year old girl comes back from school camp with a temperature, breathlessness and cough, whichhas been going on for 10 days

C. Erythromycin

Note: Outbreaks of whooping cough may in school camps. The most vulnerable children are not immunized.

3) A 5 month old baby presents with a fever, lethargy, poor feeding and a generalized convulsion. Anexamination of the cerebrospinal fluid shows an elevated number of neutrophil polymorphs

B. Cefotaxime

Note: Meningitis should be suspected in any i ll baby and may present with unusual crying, poor feeding, fever,coma, fits and vomiting. Bulging or tense anterior fontanelle, stiff neck, opisthotonus and shock may bepresent. For children more than three months old cefotaxime is the treatment of choice.

4) A 3 year old Asian girl presents with a high temperature for six days, cervical lymphadenopathy,conjunctivitis and a red tongue. There is a generalized rash, with desquamation of the skin of the fingersand toes.

E. Immunoglobulin

Note: Kawasakis disease is characterized by bilateral conjunctival injection, oral mucosal changes with redsore tongue, polymorphous rash, lymphadenopathy and desquamation of palms and soles. Inflammatorymarkers are elevated and treatment is with intravenous immunoglobulin.

5) A 4 year old girl presents with a temperature of 39oC having just had a generalized convulsion lasting3 minutes; twenty minutes later, she is completely alert again. Her older brother had similar fits at the ageof 3 years

G. Paracetomol

Note: Febrile convulsions are generalized tonic/clonic symmetrical seizures which occur with rapid rises in

temperature in children between the ages 3 months and 5 years who have no history of epilepsy or CNSdisease.

28) Theme : Fine Motor Development

A. 3 monthsB. 6 monthsC. 9 monthsD. 12 monthsE. 18 months

F. 2 yearsG. 3 yearsH. 4 yearsI. 5 yearsJ. 6 years

For each description of a child's fine motor development choose, from the list of options, thehighest development age the child is most likely to have achieved.


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1) A child can copy a square and a triangle but not yet a diamond.I. 5 years

2) A baby transfers objects to his mouth and has a fine pincer grip using thumb and forefinger.D. 12 months

3) Can only copy a circle and builds a tower of 9 cubes.G. 3 years

Comments: This question relates to the fine motor area of development.1) At 4 months the infant is able to grasp big objects and move them in to the mid-line.2) At 6 months the baby can grasp an object such a rattle and starts to transfer it from hand to hand. Heor she also starts to discover the rest of his or her body parts.3) The pincer grip develops between 6 or 9 months and by 10 months the fine pincer grip is achievedwith the baby being able to pick up small pellet like objects between thumb and forefinger.4) At 18 months the baby starts to spontaneously scribble if offered a crayon for example.5) At 24 months the child is able to copy a straight line.

6) By age 3 years the child can copy a circle.7) By age 4 years the child can copy a cross.8) By 4½ years the child can copy a square9) By 5 years of age a triangle,10) A diamond at about 6 years of age. At the age of 3 the child starts to draw people usually starting as a “head and stick” extremity person andas development progresses the person becomes more sophisticated.

29) Theme : Poisoning

A. AntipsychoticsB. BenzodiazepinesC. Ethylene glycol

D. MethanolE. Opiates

F. OrganophosphatesG. SalicylatesH. Sympathomimetic

I. Tricyclic antidepressantsJ. Volatile solvents

Select the most appropriate agent from the above list that would elicit the following toxic effects:

1) Blindness, pulmonary oedema, metabolic acidosis, shock.D. Methanol

2) Coma, hypotension, hypoventilation, reduced muscle tone and diminished reflexes.B. Benzodiazepines

3) Dysphagia, Dysphoria, Trismus, oculogyric crisisA. Antipsychotics

Comments: The relevant feature of the first case is the blindness and acidosis which is probably due to Methanol,itself a weak acid but it causes lactic acidosis and optic neuritis. The second case has features of CNSdepression and Benzodiazepines are typical. This should respond rapidly to Flumazenil. The thirdscenario with oculogyric crisis suggests antipsychotics.

30) Theme : chest pain in children

A. Acute severe asthmaB. Cardiac failureC. Costochondritis

D. Diabetic ketoacidosisE. Hysterical over-breathing

F. Inhalation of foreign bodyG. Pleura effusionH. Pulmonary embolism

I. Tension pneumothoraxJ. Unilateral pneumonia


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Select the most appropriate diagnosis from the options above that best explains the followingcases:

1) 14 year old presents to Accident & Emergency with a sudden onset of chest pain and shortness ofbreath. Examination reveals reduced expansion and hyperresonance over one side with reduced breathsounds.

I. Tension pneumothorax

2) A 17 year old presents with a sudden onset of chest pain. Examination reveals a sinus tachycardiaand a pleural rub.

H. Pulmonary embolism

3) A 13 year old boy presents with tachypnoea and tachycardia. Examination of the chest reveals poorair entry, however no crackles or wheeze. He has a pulsus paradoxus.

A. Acute severe asthma

Comments: Tension pneumothorax may result following trauma. It is a potentially life threatening event with air

accumulating under the pleura in the pleural space, which pushes the mediastinum across the chest.Children may be shocked and will be hypoxic with reduced air entry and hyperresonance. Distendedneck veins may also be visible.Pulmonary embolism is uncommon in infants and children. It may arise in children as a result of surgeryfor example scoliosis surgery. It may also occur following prolonged inactivity. In adolescents drug abuse,recent abortion or the oral contraceptive may be predisposing problems. Clinical manifestations maysuggest pneumonia. Dyspnoea is common associated with pain. There may be no physical signsalthough the embolism is sufficiently large there may be a pleural friction rub.The clue in the third case the relatively silent chest associated with pulsus paradoxus. This wouldsuggest severe asthma. OtherCauses of pulsus paradoxus include : (pericardial tamponade /effusion/ constriction and myocarditis).

31) Theme : Abdominal Disease A. Acute gastro-enteritisB. AppendicitisC. ConstipationD. Diabetes MellitusE. Henoch Schonlein Purpura

F. Mesenteric AdenitisG. NephroblastomaH. PancreatitisI. Sickle Cell crisisJ. Wilm's Tumour


1) A 9 year old Italian girl has severe abdominal pain. She is found to have sclerae and painful fingers.I. Sickle Cell crisis

Note: Sickle cell disease is a haemoglobinopathy caused by substitution of Valine for Glutamic acid at the 6thposition of the Beta chain. It results in a haemolytic anaemia resulting in vaso-occlusive episodes leading

to splenic infarction and dactylitis.

2) An 11 year old boy with a 12 hour history of anorexia and polyuria complains of severe abdominalpain. On examination he has a temperature of 38°C and is tender posteriorly on pr examination. BM is4mmol per litre.

B. Appendicitis

Note: describes acute appendicitis. The commonest acute surgical emergency of childhood. Usually seen inchildren over the age of 5. Children present with a temperature, abdominal pain and guarding in the rightiliac fossa, rebound tenderness, fever and vomiting. It is as a result of mucus obstructing the lumencausing ischaemia of the mucosa and ultimately perforation.

3) An 11 year old boy with cystic fibrosis presents with recurrent attacks of abdominal pain, vomiting and

fever.H. Pancreatitis


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Note: relates to description of chronic Pancreatitis in a child with cystic fibrosis. It is also a feature of chronichyperlipidaemia or congenital anomalies of the biliary retract or pancreas. Children present with vomitingand abdominal pain which radiates through tot he back. Clinical suspicion is confirmed by elevatedamylase levels and calcification of the pancreas on abdominal x-ray. Treatment is supportive

32) Theme : Abdominal diseases of Childhood

A. AerophagiaB. Alpha 1 antitrypsin deficiencyC. GalactosaemiaD. Hirschsprung's diseaseE. Intussusception

F. Lactose intoleranceG. Mesenteric adenitisH. Reyes syndromeI. ThalassaemiaJ. Wilson's disease

Select one of the above options that is the most appropriate diagnosis for the following cases:

1) A 10 year old child develops severe vomiting and becomes encephalopathic during a viral illness.H. Reyes syndrome

Note: Reyes syndrome is an acute disease of the liver often associated with a viral infection or Salicylatetoxicity . Laboratory findings include abnormal liver and muscle enzymes. Treatment is supportive.

2) A 3 year old child with a 2 day history of headache and pharyngitis complains of severe abdominalpain.

G. Mesenteric adenitis

Note: Mesenteric adenitis commonly accompanying upper respiratory tract infections and is due toinflammation in the abdominal lymph nodes. There is often a history of pharyngitis and abdominal pain.Examination often reveals cervical lymphadenopathy and central tenderness.

3) A 28 day old jaundice baby with early onset vomiting and diarrhoea.C. Galactosaemia

Note: Galactosaemia results as a result of galactose 1 phosphate uridyl transferase. It accumulates in kidneys,liver and brain and clinical manifestations include jaundice, hepatomegaly, hypoglycaemia, vomiting,seizures and poor weight gain. Diagnosis is made by identifying urinary galactose. Treatment is dietaryadjustment.

33) Theme : Skin lesions

A. Café au lait spotsB. Capillary haemangiomaC. Port-wine stainD. Shagreen patchE. Adenoma sebaceum

F. Cutis MarmorataG. Herald's patchH. Peri-oral pigmentationI. HypopigmentationJ. Tuber


1) A child has an abundance of birth marks associated with axillary freckling.A. Café au lait spots

Note: relates to Neurofibromatosis type 1. Café au lait lesions are brown pigmented marks found in abundancein Neurofibromatosis as well as other genetic conditions for example Tuberous sclerosis.

2) A child with learning difficulties is described as having goose like flesh.D. Shagreen patch

Note: relates to Tuberous sclerosis. It is a condition where by hamartomatous lesions become evident in earlychildhood. Seizures and learning difficulties are common and skin manifestations include fibrousangiomatous lesions in the naso labial folds known as adenoma sebaceum. White macules are seenover the trunk known as Ash leaf macules and Shagreen patches resemble goose like flesh.


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Subungual fibromata are also common.

3) A child with a history of Iron deficiency anaemia presents with per rectal bleeding and is found to havethis characteristic appearance.

H. Peri-oral pigmentation

Note:

refers to Peutz-Jeghers syndrome characterized by peri-oral pigmentation. A history of Iron deficiencyanaemia, colonoscopy may reveal polyps.

34) Theme : Personal and Social Development

A. 3 monthsB. 8 monthsC. 12 monthsD. 18 monthsE. 2 years

F. 3 yearsG. 4 yearsH. 5 yearsI. 6 yearsJ. 7 years

For each description of a child's personal and social development choose, from the list of

options, the highest development age the child is most likely to have achieved.

1) A baby shows stranger awareness, is able to wave and clap his hands and is starting to spoon feedhimself.

C. 12 months

2) Can dress and undress including buttons and can imitate the construction of bridges and steps withblocks.

G. 4 years

3) A child is starting to indicate the need to sit on the potty, play is usually solitary at this age and thechild tries to control objects over other children.

E. 2 years

Comments:This question relates to the personal and social aspects of normal development.1) Between 8- 10 months babies become anxious when separated from the main carer, usually themother for example if mother leaves the room.2) Between 9-10 months the infant is able to wave bye bye and enjoys games such as peek-a-boo.Between 18-24 months most children start to express their toilet needs.3) Up until the age of 3 plays is usually solitary, playing alongside their peers.5) By 4 years the children usually start to role play for example pretending to be the same sex parent andplay involves other children in parallel first and then interactively at around this age.

35) Theme : Genital disorders

A. Aarskog syndromeB. Fragile XC. Gonadal dysgenesisD. HermaphroditismE. Polycystic ovaries

F. Testicular feminisation syndromeG. XO syndromeH. XXX syndromeI. XXY syndromeJ. XYY syndrome

Select the most appropriate diagnosis from the list above that best describes the following cases

1) A 12 year old girl has seizures, depression and a conduct disorder.H. XXX syndrome

Note: describes a girl with XXX syndrome. Individuals are tall and thin. They have behavioural problems andare mildly dysmorphic with epicanthic folds and hypertelorism. Seizures are common


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2) A 16 year old female presents with obesity, oligomenorrhoea and hirsutism.E. Polycystic ovaries

Note: relates to Stein-Leventhal also known as polycystic ovarian syndrome. During adolescence there ischange in body habitus and there is excess hair grown and chronic and ovulation.

3) A 14 year old phenotypic female presents with primary amenorrhoea. She has normal female externalgenitalia but intra-abdominal testicles with an XY karyotype.

F. Testicular feminisation syndrome

Note: The third case has testicular feminisation, a condition associated with testosterone receptor defects thatresult in insensitivity to testosterone with resultant female genitalia.

36) Theme : NEONATAL JAUNDICE

A. Physiological jaundiceB. Biliary atresiaC. HypothyroidismD. Rhesus incompatibilityE. Congenital spherocytosis

F. Congenital cytomegalo virus infectionG. GalactosaemiaH. Glucose-6-phosphate dehydrogenase deficiencyI. Cystic fibrosisJ. Fructose intolerance

For each of the following jaundiced babies, select the most likely cause.

1) A two-week-old full term baby with a birth weight of 1.9 kg. He has gained a small amount of weightalready. On examination he has a white reflex on shining a bright light into both pupils, moderate jaundice, a pansystolic murmur at the left lower sternal edge, hepatosplenomegaly and a rash.

F. Congenital cytomegalo virus infection

Note: Low birth weight, white reflex suggesting congenital cataracts, jaundice and hepatosplenomegaly areindicative of a congenitally acquired infection. The rash is likely to be petechiae secondary to bonemarrow failure from the infection.

2) A 48 hour old baby who presents with poor feeding. She was delivered at home following a concealedpregnancy. She was thought to be pale initially but her skin subsequently became more yellow. Hermother had a late miscarriage one year previously and is blood group A negative. Investigations show ahaemoglobin of 10 g/dL and an unconjugated bilirubin of 426 mmol/L. The blood film shows evidence ofhaemolysis but otherwise normal red cell morphology.

D. Rhesus incompatibility

Note: The previous miscarriage has sensitized the maternal immune system to the Rhesus antigen.Haemolysis causes an unconjugated hyperbilirubinaemia. ABO incompatibility unlikely unless the motheris blood group O.

3) A 5 day old baby appears jaundiced but otherwise well and feeds normally. The serum bilirubin ischecked by the community midwife and found to be moderately elevated at 298 mmol/L. It is mainlyunconjugated bilirubin, and the haemoglobin is normal.

A. Physiological jaundice

Note: This is a well baby and with only a slightly elevated unconjugated bilirubin. Levels can normally increaseto 300 mmol/L with no adverse effects on the baby. Some reports say that levels are safe up to 400mmol/L in a term well baby.


A. Gastro-oesophageal refluxB. IntussusceptionC. Meconium ileusD. Meconium plug syndrome

E. Meckel's Diverticulum

F. Mid gut volvulusG. Necrotising enterocolitisH. Peptic ulcerI. Pyloric stenosis

J. Tracheo-oesophageal fistula with atresia


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Select the most appropriate option from the above list that would account for the followingpresentations:

1) A baby on the postnatal ward is seen to have a cyanotic episode during feeding.J. Tracheo-oesophageal fistula with atresia

Note: Tracheo-oesophageal fistula with oesophageal atresia results in excessive oral secretions. The baby

often chokes or coughs with feeds and becomes cyanotic. There is an inability to pass a nasogastric tubeand there is often a history of maternal polyhydramnios.

2) A baby born at 33 weeks develops vomiting and abdominal distension on day 3. By day 4 theabdomen appears erythematous and the baby becomes apnoeic.

G. Necrotising enterocolitis

Note: Necrotising enterocolitis is a life threatening condition resulting in mucosal and transmural necrosis. It ismost prevalent in low birth weight babies and the babies present with abdominal distension, gastricretention, bloody stools and signs of a sepsis. Abdominal x-ray may reveal pneumatosis intestinalis.

3) A term baby is admitted to Special Care Baby unit with hypoglycaemic episodes. His mother is apoorly controlled diabetic. By day 4 the hypoglycaemia has resolved but the baby has not yet opened his

bowels.D. Meconium plug syndrome

Note: Meconium plug syndrome is as a result of a plug of meconium causing obstruction. There is usually anunderlying disease such as cystic fibrosis, Hirschsprung's disease, infant of a diabetic mother or maternaldrug abuse.

38) Theme : Treatment of Diarrhoeal illness

A. MetronidazoleB. Gluten free dietC. No actionD. Steroids

E. Lactose free diet

F. Pancreatic enzyme supplementsG. Vitamin DH. High fibre dietI. Breast milk allergy

J. Praziquantel

Select one option from the list above that is most suitable for the following patients:

1) A 7 month old baby has diarrhoea, which mother feels has been brought on by the introduction ofweaning. Mum is a known asthmatic.

E. Lactose free diet

Note: refers to Cow's milk protein intolerance is mainly a clinical diagnosis with a history that symptomsappeared at the time of cow's milk ingestion. The disorder is usually temporary and can be managed bydietary adjustment.

2) A 13 year old Irish girl complains of diarrhoea. She is Iron deficient and denies any history of GI upset

after eating rice or potatoes. She has high anti-reticulin antibodies B. Gluten free diet

Note: refers to Coeliac disease where a gluten free diet is appropriate. High reticulin or more specifically nowanti-TTG antibodies are expected. The diagnosis is confirmed by jejunal biopsy.

3) A 10 year old boy presents with faecal urgency and bloody diarrhoea. He is also under the care ofophthalmologists for a chronic iritis.

D. Steroids

Note: relates to inflammatory bowel disease, Crohn's disease and ulcerative colitis respectively. In Crohn'sdisease : the colonoscopy will reveal cobbled stone mucosal appearances with fistulae where as inulcerative colitisI crypt abscesses are present.


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A. Cyclical vomitingB. Duodenal atresiaC. Over feedingD. Meckel's diverticulumE. Ulcerative colitis

F. Meconium ileusG. Gastro-enteritisH. Gastro-oesophageal refluxI. Pyloric stenosisJ. Urinary tract infection


1) An 8 year old girl shows signs of moderate dehydration. She has vomited all fluids of 24 hours and thevomit is not bile stained. Her abdomen is soft and she has had 3 similar episode in the past year.

A. Cyclical vomiting

Note: Cyclical vomiting. This is thought to be a variant of migraine. The child presents with bouts of vomitingwhich may persist for days and at times a headache is associated.

2) A 12 week old infant vomits after each feed. He is developmentally normal and is fed by bottle at260mls per kilo per day.

C. Over feeding

Note:

Over feeding frequently results in regurgitation and vomiting. This may also be accompanied by anexcessive weight gain and dietary adjustment is indicated.

3) An 11 year old boy with a 3 month history of abdominal pain and weight loss presents with bloodydiarrhoea.

E. Ulcerative colitis

Note: The last question relates to ulcerative colitis a form of inflammatory bowel disease. In this case the childpresents with recurrent bouts of profuse bloody diarrhoea often with mucus and abdominal pain. Associated features include arthritis, pyoderma gangrenosum, erythema nodosum and anaemia.

40) Theme : Drugs to be avoided during breastfeeding A. AmiodaroneB. AspirinC. BenzodiazepineD. ChloramphenicolE. Heparin

F. LactuloseG. LithiumH. SulfasalazineI. SulphonamidesJ. Warfarin

Which of the above drugs when prescribed to the mother is associated with the followingabnormalities in the breast-fed infant:

1) May result in neonatal hypothyroidism.A. Amiodarone

2) May result in drowsiness and failure to thrive.

C. Benzodiazepine

3) May result in neonatal diarrhoea.F. Lactulose

Comments: Administration of certain drugs to breast feeding mums may result in toxicity in the infants if thedrug enters the milk in pharmacologically significant quantities. Some drugs also inhibit thebabies sucking reflex. Other drugs inhibit lactation.

Amiodarone needs to be avoided as it may result in neonatal thyroid abnormalities as there isa theoretical risk from the release of Iodine.Benzodiazepine may render the infant drowsy resulting in poor growth.

Laxatives if used over a prolonged period may enter the breast milk resulting in the babydeveloping diarrhoea.


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41) Theme : Paediatric endocrinology

A. 3 Beta hydroxysteroid dehydrogenasedeficiencyB. 17 Hydroxylase deficiencyC. 21 Hydroxylase deficiency

D. Adrenal hyperplasiaE. Craniopharyngioma

F. HypoadrenalismG. PhaeochromocytomaH. PanhypopituitarismI. Polyglandular endocrinopathyJ. Primary aldosteronism

Select the most appropriate diagnosis from the above options to explain the followingpresentations:

1) An 8 year old boy presents with a history of enuresis. Examination reveals a bi-temporal hemianopiaand papilloedema.

E. Craniopharyngioma

Note: refers to a child with a Craniopharyngioma which is the commonest supratentorial tumour found inchildren. Children present with accelerated head growth or headaches and examination findings includepapilloedema and a bi-temporal hemianopia This tumour may result in diabetes insipidus, hypothyroidismand growth hormone deficiency.

2) A term baby is born with ambiguous genitalia. Examination reveals a phallus with pigmented labialscrotal folds and the baby is found to be hyponatraemic.

C. 21 Hydroxylase deficiency

Note: describes a baby with congenital adrenal hyperplasia. 95% of cases are due to 21 Hydroxylasedeficiency and can be diagnosed by increased levels of 17OHP. The salt wasting results inhyponatraemia (Na n) and hyperkalaemia (k G).

3) A 10 year old girl complains of headaches and vomiting. On examination she is noted to be pale andher blood pressure measures 135/90. Numerous café au lait lesions are found over her trunk.

G. Phaeochromocytoma

Note:

describes a child with Neurofibromatosis complicated by : phaeochromocytoma, which is a tumourarising from the adrenal medulla or chromaffin cells. Children present with headaches, palpitations,pallor, vomiting and night sweats. They are found to be hypertensive.

42) Theme : Drugs to be avoided during breastfeeding

A. AmiodaroneB. AspirinC. BenzodiazepineD. Chloramphenicol

E. Heparin

F. LactuloseG. LithiumH. SulfasalazineI. Sulphonamides

J. Warfarin

Which of the above drugs, when prescribed to the mother, is associated with the followingabnormalities in the breast-fed infant:

1) May result in hypotonia and cyanosis.G. Lithium

2) May result in prolonged jaundice.I. Sulphonamides

3) May result in platelet dysfunction.B. Aspirin


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Comments: Administration of certain drugs to breast-feeding mums may result in toxicity in the infants ifthe drug enters the milk in pharmacologically significant quantities. Some drugs also inhibit thebabies sucking reflex. Other drugs inhibit lactation.Lithium is also to be avoided as it may result in reduced tone and cyanotic episodes.

Aspirin may result in a possible risk of Reyes syndrome; regular use may impair platelet

function, as well as producing a hypoprothrombinaemia, especially if the infant is low inVitamin K stores.

43) Theme : Genital disorders

A. Aarskog syndromeB. Fragile XC. Gonadal dysgenesisD. HermaphroditismE. Polycystic ovaries

F. Testicular feminisation syndromeG. XO syndromeH. XXX syndromeI. XXY syndromeJ. XYY syndrome

Select the most appropriate diagnosis from the list above that best describes the following cases

1) A young child is noted to have Cryptorchidism and a shawl scrotum.A. Aarskog syndrome

Note: describes a boy with Aarskog syndrome. Features include mild – moderate learning difficulties, shortstature, facial dysmorphic features include hypertelorism anteverted nostrils and a webbed neck. Genital

abnormalities include shawl odW scrotum and cryptorchidism8 (hidden or obscure testis).

2) A 12 year old boy is noted to have a long thin face, micropenis, hypospadias and has learningdifficultie

J. XYY syndrome s.

Note: relates to a child with XYY syndrome. Individuals are usually tall and have learning difficulties andabhorrent pqrst behaviour.

3) A child is noted to have macrocephaly and macroorchidism.B. Fragile X

Note: describes a boy with Fragile X syndrome. Features include moderate learning difficulties, speech delayand autistic features. They have large heads and prominent ears as well as testicular enlargement.

44) Theme : ABDOMINAL PAIN

A. PeritonitisB. Appendicitis

C. ConstipationD. Urinary tract infectionE. Mesenteric lymphadenitis

F. MigraineG. Intussusception

H. Henoch-Schonlein vasculitisI. PsychosomaticJ. Shigella dysentry

For the following patients with abdominal pain, choose the most likely diagnosis.

1) A 7 year old girl who has felt anorexic since the previous evening and has vomited 3 times since. Shehas abdominal pain, which was made worse by the bumpy car journey to hospital.

B. Appendicitis

Note: A typical description of appendicitis. In the younger child/ baby there is often a later presentation withperforation of the appendix being relatively more common, as these children are not able to articulatetheir pain and localization of pain is more difficult to discern with examination.

2) A 4 year old boy who has a sore throat, mild fever and peri-umbilical abdominal pain.E. Mesenteric lymphadenitis


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Note: Preceding sore throat and periumbilical pain are typical of mesenteric lymphadenitis in a relatively wellchild.

3) A 9 year old boy has intermittent severe generalized colicky abdominal pain which is getting worse. He

has started to vomit over the last 24 hours. He has a history of recurrent abdominal pain. Examinationreveals a mass in the left iliac fossa.

C. Constipation

Note: This is a description of chronic severe constipation, which in extreme cases can cause obstruction. Thereis faecal loading implying the chronicity. When it is this severe it is important to rule out other pathologyand consider the possibility that is a manifestation of child abuse

45) Theme : PRECOCIOUS PUBERTY

A. CraniopharyngiomaB. Exaggerated adrenarcheC. McCune-Albright syndromeD. Hypothalamic hamartomaE. Thelarche variant

F. Neurofibromatosis type 1G. Granulosa-theca cell tumourH. HypothyroidismI. Premature thelarcheJ. Congenital adrenal hyperplasia

Which is the most likely diagnosis in the following cases:

1) A 4 year old girl present s with vaginal bleeding and café –au-lait pigmentation which stops at themidline. Subsequent investigations demonstrate gonadotrophin-independent puberty

C. McCune-Albright syndrome

Note: McCune Albright syndrome is caused by an activating mutation of the GS alpha subunit. This results inautonomous secretion of certain hormones e.g. thyroxine, cortisol, oestradiol. There may be associatedskin pigmentation and fibrous dysplasia.

2) A 3 year old girl presents with pubic hair development, cliteromegaly and acnes. There was anacceleration in her height velocity and no symptoms of oestrogen excess. Neurological examination wasnormal.

J. Congenital adrenal hyperplasia

Note: Virilising Congenital adrenal hyperplasia (CAH) typically presents with symptoms of androgen excess.There is often behavioural disturbance with mood swings. Diagnosis is confirmed by a raised 17hydroxyprogesterone. Cliteromegaly and a sustained increase in height velocity does not occur inadrenarche.

3) A 2year old girl presents with isolated bilateral breast development. Examination is otherwise normalas is her height velocity. Subsequent investigation demonstrates undetectable oestradiol levels and aprepubertal response to an LHRH test.

I. Premature thelarche

Note: There may be a slight FSH predominance on the basal gonadotrophin levels. Premature thelarche is selflimiting and thought to be the result of the body being exquisitely sensitive to low levels of oestradiol.


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A. Gastro-oesophageal refluxB. IntussusceptionC. Meconium ileusD. Meconium plug syndromeE. Meckel's Diverticulum

F. Mid gut volvulusG. Necrotising enterocolitisH. Peptic ulcerI. Pyloric stenosisJ. Tracheo-oesophageal fistula with atresia

Select the most appropriate option from the above list that would account for the followingpresentations:

1) 5 week old breast fed baby is admitted with a 2 week history of vomiting and dehydration. Onexamination an olive sized mass is palpable.

I. Pyloric stenosis

Note: Pyloric stenosis is most prevalent amongst first born male and age of onset is usually between 3-6 weeksof age. Babies present with projectile vomiting and examination may reveal visible peristaltic waves andan olive sized mass may be palpable in the upper epigastrium.

2) A 3 month old baby boy is admitted with episodic screaming. On examination he is mottled and has adelayed capillary refill time. Palpation of the abdomen reveals a sausage shaped mass.

B. IntussusceptionNote: Intussusception – a segment of bowel becomes telescoped in to an adjacent of bowel often in areaswhere the bowel is aperistaltic, for example Peyer's patches, Meckel's diverticulum, tumour or an area ofoedema secondary to Henoch Schonlein purpura. The child presents with pain, vomiting and signs ofshock. A mass may be palpable in the right upper quadrant.

3) A baby with cystic fibrosis develops vomiting and abdominal distension. A ground glass appearance isseen on x-ray.

C. Meconium ileus

Note: Meconium ileus syndrome is the commonest form of small bowel obstruction and the baby presents withdistension, vomiting and constipation. An x-ray may reveal a ground glass appearance.

47) Theme : Gross motor development

A. 1 monthB. 2 monthsC. 4 monthsD. 6 monthsE. 8 months

F. 10 monthsG. 1 yearH. 18 monthsI. 2 yearsJ. 3 years

For each description of a child's gross motor development choose, from the list of options, thehighest development age the child is most likely to have achieved.

1) A baby has good head control with no head lag when pulled from supine to sitting.

C. 4 months

2) A baby can rise independently and stands momentarily alone. Is starting to take 1-2 steps.G. 1 year

3) A child who is walking and runs stiffly can climb stairs if hand is held.I. 2 years

Comments: This question tests your knowledge of the development stages of gross motor skills.

1) At 3 months when placed prone an infant can lift his head with his arms extended.2) By 4 months head lag is lost and this is evident when raised from supine to the sitting position.

3) At 5 months the baby starts to roll over.4) At 6 months the baby is starting to sit up with support for example cushions.


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5) Sitting unsupported is usually seen by 6-7 months and6) At 9 months the baby can start to crawl.7) Cruising is usually achieved by about 10 months.8) At 12 months the baby can rise independently and often starts to take a few steps alone.9) At 18 months the toddler can run although this is usually in a tiff fashion. He or she can ascend

stairs with assistance.10) At 20 months the child can climb the stairs holding on to a rail.

11) At 3 the child can ascend stairs in an adult fashion using alternating feet per step, however isunable to descend the stairs in such a fashion until 4 years of age.

48) Theme : SHORT STATURE

A. Constitutional delay of growthB. Growth hormone deficiencyC. Psychosocial deprivationD. Familial short statureE. Skeletal dysplasia

F. HypothyroidismG. CraniopharyngiomaH. Turner’s syndromeI. Cushing’s syndrome

Which is the most likely diagnosis in the following cases?

1) A previously abused 4 year old child presents with growth failure over the last 12 months. She was

placed in foster care for the last 18 months and is said to much happier. Her height velocity has sloweddown and she has gained weight. She suffers with constipation and her teacher has noticed her attentionspan has got worse recently.

F. Hypothyroidism

Note: Height velocity will improve once abuse is removed. Constipation and poor attention span are commonsymptoms of hypothyroidism in children.

2) A 7 year old boy was referred to clinic for investigation of short stature. He is well and apart fromoccasional headaches has no other complaints. His height is below the 0.4 th centile and within thetarget centile range calculated from his parents heights. Previous height measurements from his GPshow no evidence of a reduction in height velocity.

D. Familial short stature

Note: Short stature with no evidence of growth failure and a height within the target centile range indicatefamilial short stature which requires no further investigation.

3) A 3 year old girl presents with stature well below the 0.4 th centile. She had a past history of pedaloedema at birth and uncomplicated repaired coarctation of the aorta. She has been treated forconstipation in the past but this has resolved, other wise she is very well. An insulin tolerance testdemonstrates a peak growth hormone level of 25 mU/l.

H. Turner’s syndrome

Note: The insulin tolerance test should only be performed if there is growth data that suggests growth failureand other causes of short stature have been ruled out. GH deficiency is not suggested here. She is veryshort and also well i.e. other pathology unlikely. A previous history of coarctation and pedal oedemasuggests Turner’s syndrome.

49) Theme : Childhood malignancies

A. Burkitt's nasopharyngeal carcinomaB. Ewing sarcomaC. HepatoblastomaD. Hodgkin's lymphomaE. Melanoma

F. NesidioblastosisG. NeuroblastomaH. TeratomaI. Wilm's tumourJ. Xeroderma pigmentosum

Select the most appropriate oncological diagnosis from the above list of options to explain thefollowing scenarios.


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1) A baby is born with a solitary mass over the right buttock.H. Teratoma

2) A young child presents with fevers and a mass in the femur is noted and a diagnosis of malignancy ismade. This malignancy is 30 times more common in white children compared to black children.

B. Ewing sarcoma

3) A child of Nigerian parents presents with a unilateral tender cervical node. A diagnosis of malignancyis made with this malignancy being 7 times more common in Black Children compared with Whitechildren.

A. Burkitt's nasopharyngeal carcinoma

Comments: Item 1 relates to a baby born with a sacrococcygeal teratoma. A solid tumour found in new-borns withgirls being affected more than boys. It is a mixed germ cell tumour and may be associated withgastrointestinal or genital urinary symptoms. Ewing sarcoma is a tumour of the bone, usually found inlong bones particularly the femur. It may present with pain, fever and tenderness and sometimes

pathological fractures. There is a poor prognosis. It is 30 times more common in white children than blackchildren. This genetic predisposition is contrary to the Burkitt's nasopharyngeal carcinoma which is morecommon in black children than white (by 7 fold). This tumour has been associated with the Epstein-Barrvirus and children present with sore throats, unilateral tenderness, cervical lymphnodes, trismus andweight loss.

50) Theme : Cranial Nerves

A. AbducensB. AuditoryC. FacialD. GlossopharyngealE. Oculomotor

F. OlfactoryG. OpticH. TrochlearI. TrigeminalJ. Vagus

Which of the above cranial nerves is affected in the following cases?

1) A 3 year old girl recovering from a pneumococcal meningitis complains of a painful right eye. Thepupil is enlarged and the eyes deviated inferiorly and laterally. There is a right sided ptosis.

E. Oculomotor

2) A 12 year old boy complains of a numb feeling to the left side of his face. He is unable to whistle orshow is teeth on that side.

C. Facial

3) A 15 year old girl on treatment for Nephrotic syndrome presents with a vesicular rash involving theright conjunctiva and right forehead, which is very tender to touch.

I. Trigeminal

Comments: The oculomotor nerve innovates the superior inferior and medial rectus as well as the inferior obliqueand the levator palpebrae muscles. Complete paralysis of the oculomotor nerve causes ptosis, dilatationof the pupil and displacement of the eye outwards and downwards. An upper motor neurone facial nerve palsy is diagnosed by decreased voluntary movement of the lowerface with flattening of the nasal folds, where as a lower motor neurone lesion tends to involve the upperand lower facial muscles.The Trigeminal nerve – sensory distribution to the face is divided in to ophthalmic, maxillary and themandibular routes and motor function may be tested by examining the masseter and temporalis muscles.


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51) Theme : Causes of Headaches

A. Benign intra-cranial hypertensionB. Cerebral tumourC. Cluster headacheD. EncephalitisE. Pseudotumour Cerebri

F. MeningitisG. MigraineH. Subdural haemorrhageI. Sub arachnoid haemorrhageJ. Tension headache

Select the most appropriate diagnosis from the above list that would explain the following cases.

1) A 6 year old girl with a history of Still's disease presents with a 2 month history of headache anddiplopia She is admitted to hospital. Investigations carried out include a normal CT scan, normal CSFprofile and normal opening pressures.

E. Pseudotumour Cerebri

2) A 4 month old infant presents with poor feeding and irritability. On examination he has a torticollis andhis head circumference has increased form the 50th – 98th centile since his last measurement 6 weeksago.

3) A 16 year old obese girl with a long history of headaches is found to have papilloedema. The rest ofthe neurological examination is normal as is the CT scan.

A. Benign intra-cranial hypertension

Comments: Intra-cranial tumours- brain tumours are second to leukaemia as the most prevalent malignancy inchildhood. Children present with either signs of increased intra-cranial pressure or with focal neurologicalsigns. Infratentorial tumours are more common in the paediatric age group than supratentorial tumoursand the MRI scan is the best radiological tool for delineating brain tumours. Pseudotumour cerebri is aclinical syndrome mimicking brain tumours and characterised by normal CSF pressure and cell count and

a normal structure to the brain. Causes of which are multiple for example metabolic disorders, infections,drugs, haematological disorders. Treatment focuses towards the underlying aetiology and pseudo tumourcerebri is mainly self limiting. Benign intracranial hypertension is associated with pregnancy as well asdrugs such as the oral contraceptive, tetracyclines and rarely Growth Hormone therapy. It is morecommon in association with obesity. LP usually reveals high opening pressures and treatment includesremoval of any precipitant with possible serial LP and removal of CSF.

52) Theme : Causes of abdominal pain

A. Acute appendicitisB. Inflammatory bowel diseaseC. Irritable bowel syndromeD. Meckel's diverticulumE. Muscle strain

F. Ovarian cystsG. Pelvic inflammatory diseaseH. Psoas haematomaI. PyelonephritisJ. Ureteric calculus

Select the most appropriate diagnosis from the above list for the following presentations

1) A 13 year old boy with umbilical pain for the last 4 days presents with anorexia, nausea and has notpassed a bowel motion for 24 hours.

A. Acute appendicitis

Note: The pain of acute appendicitis starts off as a peri-umbilical pain which then locates to the right iliac fossaand is some times referred to the back. It is sharp and steady; it results in nausea, emesis and localtenderness.

2) A 10 year old boy with lower abdominal pain for the last 10 days presents with a history of passing 6-8loose stools. Temperature is 38.8°C. He is tender in the right lower quadrant and has an anal fistula.

B. Inflammatory bowel diseaseNote:


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Inflammatory bowel disease results in recurrent lower abdominal pain, often radiating to the back. Thepain is dull and crampy in nature and is associated with fever, weight loss and tenesmus.

3) A 14 year old haemophiliac patient presents with lower abdominal pain and a limp. Attempts tostraighten his leg results in excruciating pain

H. Psoas haematoma

Note: Bleed into the Psoas muscle which may be due to coagulopathy or secondary to sepsis (DIC) results inback pain radiating to the hip with painful flexion at the hip joint.

53) Theme : NORMAL DEVELOPMENT

A. 4 weeksB. 2 monthsC. 7 monthsD. 10 monthsE. 12 months

F. 18 monthsG. 2 yearsH. 3 yearsI. 4 yearsJ. 5 years

At what age would you expect the average child to achieve the following milestones:-1) Show casting, able to pick up small objects with a neat pincer grasp and have developed concept ofobject permanence.

E. 12 months

2) Able to skip, draw a triangle from copy, name the heavier of 2 weights, count up to ten and askquestions about meaning of words.

J. 5 years

3) Able to identify coloursH. 3 years

Comments:

It is important to be familiar with the developmental milestones, particularly up to the age of 3 years.Milestones are grouped under 4 headings:

• Gross motor

• Fine motor

• Hearing and language

• social

You should know the age range for each milestone. Developmental milestones are in Illingworth: 'TheNormal Child'.

54) Theme : Chemotherapy

A. 6-MercaptopurineB. Actomyosin DC. AsparaginaseD. BleomycinE. Cisplatin

F. CyclophosphamideG. Cytosine arabinosideH. DoxorubicinI. MethotrexateJ. Vincristine

Select the most appropriate chemotherapeutic agent from the above list that corresponds withthe following actions and side effects:

1) An alkylating agent resulting in haemorrhagic cystitis.F. Cyclophosphamide

2) A drug inhibiting protein synthesis may result in a dose related Pancreatitis.C. Asparaginas


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3) An anthracycline with a serious risk of cardio-toxicity.H. Doxorubicin

Comments:All cytotoxic agents have adverse effects. General side-effects include nausea,

vomiting and bone marrow suppression, alopecia and stomatitis. More specific side-

effects are listed in the questions above. Alkylating agents such as Cyclophosphamide

may cause a haemorrhagic cystitis. Asparaginase results in a dose related Pancreatitis.

The Anthracyclines e.g. (Doxorubicin and Daunorubicin) are cardiotoxic, which is

often very difficult to detect. Monitoring with echocardiograms is advised.

Vincristine is an alkaloid agent and results in sensory motor neuropathy with long-

term use. It also has an affect on the autonomic system resulting in severe constipation

and paralytic ileus. It may also result in sensory changes with parathesis progressing to

loss of tendon reflexes.

Bleomycin is the main drug resulting in lung damage and occurs in up to 10% of patients. The damage is dose related.

55) Theme : SYNDROMES

A. Down's syndromeB. Turner's syndromeC. Noonan's syndromeD. Klinefelter's syndromeE. Treacher-Collins syndrome

F. Marfan's syndromeG. Sotos syndromeH. Klippel-feil syndromeI. Laurence Moon Biedl syndromeJ. MCune Albright syndrome

Which is the most applicable diagnosis for the following children?

1) A boy with learning difficulties and short stature is found to have pulmonary stenosis when investigatedfor a murmur. He has some dysmorphic features including wide carrying angle of the arms.C. Noonan's syndrome

Note: Children with Noonan’s syndrome have short stature and may have delayed puberty. There is alsoassociated short sightedness. Pulmonary stenosis is the most frequent congenital heart lesion.

2) Boy is seen in cardiology clinic for investigation of a murmur heard on routine check. His father died atan early age from aortic dissection. He is noted to be above the 98th centile in height for his age.

F. Marfan's syndrome

Note: Marfans syndrome is also associated with a high arched palate, arachnodactly and a predisposition topneumothoraces.

3) A child with learning difficulties was noticed to be long at birth. He has a large head, downslantingeyes, bossed forehead and prominent jaw. He is clumsy and previously been investigated for hypotonia.

G. Sotos syndrome

Note: This is a typical description of Sotos syndrome which is one of the overgrowth syndromes.


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Haematochezia (bright red blood per rectum)

58) Theme : Causes of headache and fever

A. Bacterial meningitis

B. Congenital rubellaC. Congenital toxoplasmosisD. Herpes simplex encephalitisE. Leukaemic infiltration

F. Meningococcal meningitis

G. Pneumococcal meningitisH. Status epilepticusI. Tuberculous meningitisJ. Varicella cerebellitis

Select the most likely diagnosis from the above list that would account for the followingpresentations

1) A child is admitted with alternating levels of consciousness and a high temperature. EEG showsperiodic, lateralised epileptiform discharges.

D. Herpes simplex encephalitis

2) A baby is born at term weighing 2.5kilos. He is noted to have microcephaly. An ultrasound scan of hishead reveals calcification.

C. Congenital toxoplasmosis

3) A baby of 6 months has a full septic screen. His CSF shows 10 white cells predominantlylymphocytes, high protein and low glucose.

I. Tuberculous meningitis

Comments: Examination of the cerebral spinal fluid is essential in confirming the diagnosis of conditions such asmeningitis, encephalitis and sub arachnoid haemorrhage. The main contrai

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