1999 FRACP Written Examination Paediatrics & Child

1999 FRACP Written Examination
Paediatrics & Child Health
Paper 2 – Clinical Applications
Introduction
Format
Paper 1 – Medical Sciences: 70 questions; time allowed: 2 hours

Paper 2 – Clinical Applications: 100 questions; time allowed: 3 hours
All questions are in the A-type multiple-choice format, that is, the single best answer of the five options given.
In the questions, values appearing within [ ] refer to normal ranges.
When visual material has been turned on its side, an arrow on the page indicates the orientation of the visual
material.
Questions do not necessarily appear in the order in which they were first printed.
Answers
A table of answers is located at the end of each paper.
Scoring
A correct answer will score one mark and an incorrect answer zero. There is no negative marking in the FRACP
Written Examination.
Queries
Contact the Executive Officer, Examinations’ Section, Department of Training and Assessment via e-mail:
exams@racp.edu.au.
Please note that with changes in medical knowledge, some of the information may no longer be current.
Copyright © 2003 by The Royal Australasian College of Physicians
All Written Examination papers are copyright. They may not be reproduced in whole or part without written
permission from The Royal Australasian College of Physicians, 145 Macquarie Street, Sydney, Australia.
2 P299
Question 1
A 30-year-old mother and her five-year-old son are both HIV (human immunodeficiency virus)-positive. She is well
and takes all her own medication reliably. She is pregnant and would like to know what are the chances of her
baby developing HIV infection. She understands that both baby and she will be treated with the best available
current treatments.
The risk of the baby acquiring HIV is closest to:
A. 90%.
B. 70%.
C. 50%.
D. 30%.
E. 10%.
Question 2
An antenatal ultrasound reveals a right-sided thoracic lesion which appears to have a systemic blood supply. A
computerised tomography (CT) scan performed after birth reveals an abnormal right lower lobe with air-filled cysts
of varying size. Thickening and collapse are present within the same areas. Except for a cough the child has
been asymptomatic but breath sounds are reduced in this area.
Which of the following is the most likely diagnosis?
A. Bronchogenic cysts.
B. Congenital lobar emphysema.
C. Cystadenomatoid malformation.
D. Polyalveolar lobe.
E. Pulmonary sequestration.
Question 3
The following results are obtained from a two-day-old male infant.
IgG 6.53 g/L [5.34-16.94]

IgA <0.10 g/L [0-0.07]
IgM <0.09 g/L [0-0.18]
IgE <5 kU/L [<25]
Haemoglobin 189 g/L [145-225]
12
Red cell count 5.06 x 10 /L [4.00-6.60]
9
Platelet count 326 x 10 /L [150-400]
9
White cell count 11.5 x 10 /L [5.0-21.0]
Differential:
9
band forms 0.68 x 10 /L (6%)
9
neutrophils 9.01 x 10 /L (78%)
9
lymphocytes 0.10 x 10 /L (1%)
9
monocytes 1.37 x 10 /L (12%)
9
eosinophils 0.34 x 10 /L (3%)

3 P299
Question 3 (continued)
These findings are most consistent with which one of the following?
A. IgA deficiency.
B. Kostmann syndrome.
C. Normal results.
D. Severe combined immune deficiency.
E. X-linked agammaglobulinaemia.
Question 4
A 30-month-old boy presented five weeks ago with sudden onset of limp and fever, having been well previously.
Both knees were red and swollen, but rapidly improved without treatment and in 24 hours were back to normal.
His fevers persisted.
He developed an intermittent maculopapular rash over his trunk and thighs. He was often miserable and disliked
being handled, particularly being touched on his neck.
°
On examination he is miserable, febrile (39 C), has a few small lymph nodes in the neck and groin and his spleen
can be tipped. Examination is otherwise completely normal.
Investigations:
haemoglobin 103 g/L [110-140]

9
white cell count 17.9 x 10 /L [4-11]
9
platelet count 641 x 10 /L [150-400]
ESR 38 mm/h
chest X-ray moderate cardiomegaly
cardiac ultrasound moderate cardiac effusion without tamponade
Which one of the following is the one most likely diagnosis?
A. Coxsackie virus infection.
B. Kawasaki disease.
C. Rheumatic fever.
D. Systemic juvenile chronic arthritis.
E. Systemic lupus erythematosus.

4 P299
Question 5
A previously well eight-year-old boy presents to his paediatrician for evaluation of episodes occurring on a daily
basis. The episodes are brief, lasting up to one minute, and consist of the sudden appearance of "small, bright
multicoloured balls" on the left side of his visual field. Headaches occasionally follow the attacks. He has a history
of a single convulsive seizure in sleep. Neurological examination is normal.
The most likely diagnosis is:
A. benign occipital seizures.
B. cerebral tumour.
C. hysteria.
D. migraine.
E. raised intracranial pressure.
Question 6
You are called to see a four-year-old girl with seal-like barking cough, severe inspiratory stridor, marked wheeze
and agitation. Her oxygen saturation is 89% in room air.
Which one of the following would be the most appropriate immediate therapy in addition to oxygen?
A. Nebulised adrenalin.
B. Nebulised ipratropium.
C. Nebulised salbutamol.
D. Nebulised steroids.
E. Oral steroids.
Question 7
You are asked to review a baby boy who had an "unrecordable" dextrostix at three and a half hours of age after a
difficult delivery. He was born at 38 weeks gestation with a birthweight of 3370 g. Formal plasma glucose was 0.2
mmol/L. His liver was palpable three centimetres below the costal margin. The rest of the examination, including
his genitalia, was normal.
After treatment with intravenous dextrose at 5 mg/kg/min, glucose levels remained stable at 4 to 5 mmol/L, after
which the infusion was stopped and breastfeeding was started. A fasting study was performed at six days of age.
Plasma glucose at three hours post feed was 1.8 mmol/L, at 3.5 hours it was 1.2 mmol/L. Ketones were present in
the urine.
Which one of the following diagnoses is most likely?
A. Congenital adrenal hyperplasia.
B. Glycogen storage disease.
C. Hyperinsulinism.
D. Hypopituitarism.
E. Medium chain acyl co-A deficiency.

5 P299
Question 8
A six-week-old infant weighing 3300 g is referred for evaluation of tachypnoea and failure to thrive. He was born at
term weighing 3000 g. He is tachypnoeic, mildly cyanosed and has tachycardia with normal volume pulses.
Ejection and mid diastolic murmurs are heard. His transcutaneous oxygen saturation is 87% and two-dimensional
Doppler echocardiography indicates double inlet left ventricle (single ventricle) with unrestricted pulmonary and
aortic outflows.
Which one of the following would be the most appropriate surgical management?
A. Bidirectional Glenn shunt (superior vena cava to right pulmonary artery anastomosis).
B. Modified Blalock shunt (systemic to pulmonary artery anastomosis).
C. Modified Fontan procedure (total cavopulmonary connection).
D. Pulmonary artery banding.
E. Ventricular septation.
Question 9
An 18-month-old male with known sickle cell disease presents to the Emergency Department with marked pallor
developing over a day. Physical examination shows a temperature of 37.5°C, heart rate of 200/minute, blood
pressure 50/20 mmHg and cool peripheries. Abdominal examination shows moderate distension with a spleen
palpable 8 cm below the costal margin. He is slightly drowsy and irritable but responsive to verbal commands.
A. aplastic crisis.
B. cerebrovascular accident.
C. salmonella septicaemia.
D. splenic sequestration.
E. pneumococcal septicaemia.

6 P299
Question 10
In the pedigree shown above, III:3 is a woman of normal intelligence. She has a family history of mental
retardation. Her nephew IV:1 and her cousin III:1 have recently been diagnosed with Fragile X mental
retardation.
The risk that she is a Fragile X carrier is closest to:
A. 100%.
B. 50%.
C. 25%.
D. 1%.
E. nil.
Question 11
A nine-month-old baby girl presents with fever to 39.2°C for two days, and irritability. She is tender in the right
flank. Urine microscopy on a suprapubic sample reveals >100 white blood cells per high power field.
What would be the most appropriate antibiotic(s) with which to commence treatment?
A. Intravenous ampicillin and gentamicin.
B. Intravenous cefotaxime.
C. Intravenous trimethoprim-sulfamethoxazole.
D. Oral amoxycillin-clavulanic acid.
E. Oral trimethoprim-sulfamethoxazole.

7 P299
Question 12
A four-year-old boy presents with a four-week history of screaming out in his sleep at approximately 11 p.m.each
night. When his parents attend to him he is usually sitting up in bed, eyes wide, highly agitated and muttering to
himself in a frightened way. His hair is dishevelled, he is breathing quickly and his arms and legs are shaking. He
does not appear to recognise his parents and is not consoled by their presence. On several occasions the boy
has got up from his bed and run screaming from the house into the neighbours' front yard in a highly agitated
state. On two occasions he has stood and urinated in the corner of his bedroom. His agitation usually subsides
after 10-15 minutes, following which the boy seems to settle back into a peaceful sleep.
The parents consult you, as their family paediatrician, about this behaviour.
The most appropriate initial management would be:
A. carbamazepine.
B. imipramine.
C. psychiatric referral.
D. reassurance that this behaviour will settle without treatment.
E. scheduled waking of the child at 10 p.m.
Question 13
You are asked to review a 15-year-old boy regarding his short stature. He was treated for medulloblastoma at age
six with cranio-spinal irradiation. His height was on the 50th percentile at diagnosis. At age 11, when puberty was
first noticed, his height was 140 cm (25th percentile). He is now 156 cm tall and his arm span is 167 cm. His
father's height is 172 cm and his mother's height is 158 cm. Preliminary investigations include:
bone age 15 years

free thyroxine (free T4) 9 pmol/L [8-18]
thyroid-stimulating hormone (TSH) 9 mU/L [<4]
insulin-like growth factor 1 (IGF-1) 15 pmol/L [20-60]
The major cause of his short stature is:
A. attenuated pubertal growth spurt.
B. attenuated spinal growth.
C. familial short stature.
D. growth hormone deficiency.
E. hypothyroidism.
Question 14
A nine-year-old boy is referred for assessment. The school is concerned by his distractibility and disruptive
behaviour in class. He is a "loner" in the playground although he will, at times, play with five to six year olds. He
likes routine and becomes tearful if his environment changes. Cognitive assessment shows him to be of average
intelligence with verbal skills better than performance skills. Often he does not look at you when he talks.
His motor milestones have all been slightly delayed and he shows no interest in sport or physical activities. His
first words were spoken at 12 months and he was speaking in sentences at three years. He is extremely
knowledgeable about computers and bores people with his repetitive conversations about them.
Physical examination shows no specific abnormality.

8 P299
A. Asperger's disorder.
B. attention deficit hyperactivity disorder (ADHD).
C. autism.
D. depression.
E. Fragile X syndrome.
Question 15
The most appropriate treatment for moderate hypertension following post-infectious glomerulonephritis is:
A. amiloride.
B. atenolol.
C. captopril.
D. chlorothiazide.
E. frusemide.
Question 16
A 10-year-old girl is diagnosed with type 1 (insulin-dependent) diabetes mellitus. At time of diagnosis, which one
of the following antibodies is most likely to be found in her serum?
A. Adrenal antibody.
B. Gliadin antibody.
C. Glutamic acid decarboxylase antibody.
D. Insulin auto-antibody.
E. Thyroid microsomal antibody.

9 P299
Question 17
A woman develops chickenpox at 38 weeks gestation. Fourteen days later she delivers a full-term baby boy
vaginally. The baby weighs 3200 g and is covered in vesicular spots. His photo is shown below. He is clinically
well and requires no resuscitation. Physical examination is otherwise normal.
What is the most appropriate management of this baby?
A. Give intravenous acyclovir.
B. Give oral acyclovir.
C. Give intramuscular zoster immune globulin.
D. Give intramuscular zoster immune globulin and intravenous acyclovir.
E. No treatment.
Question 18
A three-year-old girl is seen in the Emergency Department with obvious jaundice and moderate pallor.
Examination reveals the presence of jaundice, no hepatomegaly but a spleen palpable 3-4 cm below the costal
margin. Full blood count shows:

mean corpuscular volume 80 fL [75-90]
9
9
white blood count 10.1 x 10 /L with
normal differential [5-14.5]

10 P299
Peripheral blood smear shows spherocytosis as shown below.
The next most appropriate test is:
A. Coombs direct antiglobulin test.
B. haemoglobin electrophoresis.
C. Hamm test.
D. haptoglobin measurement.
E. osmotic fragility test.
Question 19
A six-day-old breastfed infant has frequent watery stools for two days. Stool tests reveal a few fat globules and
fatty acid crystals, normal tryptic activity and 2% total and 2% reducing sugar. He had a temperature of 38°C on
day four and initially fed poorly.
Which one of the following is the most likely diagnosis?
A. Glucose-galactose malabsorption.
B. Hereditary fructose intolerance.
C. Primary alactasia.
D. Secondary lactose intolerance.
E. Sucrase-isomaltase deficiency.

11 P299
Question 20
A two-year-old boy is seen in the Emergency Department. He has multiple small bruises of various ages on his
limbs. He is not on medication. A maternal uncle was said to have bled easily but there was no definite family
history of any known bleeding disorder.
Physical examination confirmed bruising without any other additional abnormalities noted. Full blood count was
normal. The following coagulation results were obtained:
prothrombin ratio 1.1 [1.0-1.2]

activated partial thromboplastin time (APTT) 65 seconds [25-39]
An APTT mixing study using one part patient plasma plus one part normal plasma is found to be 63 seconds.
A. haemophilia A (classical haemophilia).
B. haemophilia B (Christmas disease).
C. Lupus anticoagulant.
D. non-accidental injury.
E. Von Willebrand's disease.
Question 21
A four-year-old girl developed a sore throat with difficulty swallowing, dribbling, fever to 39°C, and puffiness of both
upper eyelids. She was given oral penicillin V by her local doctor, but her symptoms persisted and she developed
a generalised, fine, discrete, macular rash on her trunk and face.
Examination showed a fever of 39.5°C, exudative tonsillitis, the macular rash, bilateral upper eyelid oedema, and
generalised cervical, axillary and inguinal lymphadenitis. Her spleen could be tipped. Her chest was clear. Her
photograph is shown below.

12 P299
Investigations show:

9
9
monospot negative
urinalysis 1+ protein
serum antistreptolysin-O titre (ASOT) 479 [<200]
Which of the following is the most likely diagnosis?
A. Adenovirus infection.
B. Epstein-Barr virus infection.
C. Herpes simplex virus infection.
D. Penicillin allergy.
E. Scarlet fever.
Question 22
Which one of the following is most likely to increase the risk of hepatotoxicity due to sodium valproate?
A. Increasing age of the patient.
B. Increasing the dose of valproate.
C. Polypharmacy.
D. Poorly controlled seizures.
E. Renal impairment.
Question 23
A three-day-old term neonate weighing 3100 g has clinical features of Down syndrome. She has no respiratory
distress and no apnoea. The pulses are normal, no murmur is heard and the second heart sound is loud.
Transcutaneous oxygen saturations fall intermittently to the high 80s. Two-dimensional Doppler echocardiography
shows complete atrioventricular septal defect.
Which one of the following would be the most appropriate?
A. Captopril.
B. Fluid restriction.
C. Frusemide.
D. Oxygen.
E. No intervention at this stage.

13 P299
Question 24
Inhaled nitric oxide is usually delivered into the ventilation circuit close to the patient's endotracheal tube.
This is in order to:
A. allow delivery of optimal concentrations of nitric oxide.
B. avoid exposure of nitric oxide to high humidification.
C. lessen the rate of generation of methaemoglobin.
D. minimise the reduction in FiO2 consequent on nitric oxide delivery.
E. minimise the risk of pulmonary toxicity due to other oxides of nitrogen.
Question 25
A six-year-old boy, previously well, has a one-week history of rhinitis followed by cough and fever. His chest X-ray
is shown below.
The abnormality shown is most likely to be due to which one of the following?
A. Hamartoma.
B. Metastatic lesion.
C. Neuroblastoma.
D. Pneumonia.
E. Tuberculosis.

14 P299
Question 26
A blood spot newborn screening test on a four-day-old infant revealed a high thyroid-stimulating hormone (TSH)
level. At 10 days of age this infant's serum thyroid function showed:
free thyroxine (free T4) 8 pmol/L [11-23]

TSH 520 mU/L [<8]
A thyroid scan was performed and is shown below.
The uptake of technetium isotope was 40% [2-6].
Which one of the following is the most likely cause?
A. Athyrotic hypothyroidism.
B. Exposure of the fetus to radioactive iodine in the first trimester.
C. Maternal lithium therapy in pregnancy.
D. Thyroid organification defect.
E. Untreated maternal Graves' disease.
Question 27
An intelligent eight-year-old boy has a long history of hyperactivity, impulsiveness and distractibility, together with
motor tics (blinking, facial grimaces and snorting). He has been diagnosed as having Attention Deficit
Hyperactivity Disorder (ADHD) and Tourette's disorder. He showed little response to clonidine (now ceased), but
his hyperactivity improved with methylphenidate. He now presents with a six-month history of severe anxiety. He
is unable to enter an empty room, cannot sleep alone and clings to his mother. At school he is functioning
reasonably well in academic and social areas.
At interview, he presents as tense and anxious and describes fears of monsters and dinosaurs entering his house
and attacking him. He understands that these fears are baseless but cannot stop himself from worrying. There
are no major precipitating events. You have commenced a behaviour modification program which is only partially
successful and you decide to supplement methylphenidate with another medication.

15 P299
Which one of the following medications is the most appropriate?
A. Imipramine.
B. Fluoxetine.
C. Haloperidol.
D. Moclobemide.
E. Oxazepam.
Question 28
A general practitioner requests your advice about a six-year-old boy who has sustained a laceration which has
been heavily soiled with manure after a fall in a horse stable. His immunisation status, confirmed by his child
health records, is as follows:
diphtheria-tetanus-pertussis vaccine (DTP) at two and four months

oral polio vaccine (OPV) at two and four months
measles-mumps-rubella vaccine (MMR) at 12 months
Which one of the following should the child now be given?
A. Childhood diphtheria and tetanus toxoids (CDT), tetanus immunoglobulin and OPV.
B. DTP.
C. DTP and OPV.
D. DTP, tetanus immunoglobulin and OPV.
E. Tetanus toxoid and tetanus immunoglobulin.
Question 29

16 P299
A 10-day-old male infant, born at term with a birthweight of 2400 g is lethargic and feeding poorly. There has been
no weight gain. An abdominal ultrasound demonstrates bilateral hydronephrosis. Urine output appears to be
satisfactory. The micturating cysto-urethrogram is shown (see previous page). An indwelling catheter is left in
situ.
The following biochemistry results are obtained the following morning:
Serum:
sodium 126 mmol/L [132-145]
potassium 6.9 mmol/L [3.5-6.0]
chloride 88 mmol/L [100-110]
HCO3 17.6 mmol/L [18.0-26.0]
urea 15.5 mmol/L [1.4-4.6]
creatinine 0.126 mmol/L [0.015-0.055]
albumin 26 g/L [23-46]
Urine:
sodium 68 mmol/L
potassium 24 mmol/L
osmolality 195 mOsm/Kg
The primary objective in management over the next 24 hours would be which one of the following?
A. Albumin replacement.
B. Bicarbonate replacement.
C. Potassium correction.
D. Sodium replacement.
E. Water restriction.
Question 30

17 P299
A three-year-old boy presents with obesity, mild developmental delay and bilateral post axial polydactyly.
Corrective surgery to hands and feet was carried out in the first year. His photograph is shown (see previous
page). Ophthalmological assessment shows visual acuity is normal at 6/6 in both eyes. There is a pigmentary
retinopathy and the electroretinogram is abnormal.
The most likely diagnosis to explain these findings is:
A. Bardet Biedl syndrome.
B. Fragile X syndrome.
C. hypothyroidism.
D. Prader-Willi syndrome.
E. Usher syndrome.
Question 31
An eight-year-old boy presents with behavioural problems and a noted deterioration in his performance at school.
Examination reveals mild unsteadiness of gait and a slight brown discolouration of the gums. A computed
tomography (CT) scan of his head is shown below.
Which one of the following is most likely to establish a diagnosis?
A. Magnetic resonance imaging (MRI) scan of the brain.
B. Ophthalmological examination.
C. Plasma very-long chain fatty acid ratio.
D. Serum lead level.
E. White cell lysosomal enzymes.

18 P299
Question 32
A four-year-old child presents to the emergency department with a two-day history of multiple bruises and a
bleeding nose, two weeks after an upper respiratory tract infection. His past medical history is unremarkable. On
examination, in addition to the features described in the history, there is a widespread petechial rash noted mainly
over the trunk but there are no other abnormal features. Full blood count shows the following results:

red cell morphology normal
9
platelet count <10 x 10 /L [150-400]
9
white cell count 9.8 x 10 /L [5.0-14.5]
differential:
9
lymphocytes 5.8 x 10 /L [1.5-10.0]
9
neutrophils 3.8 x 10 /L [1.0-8.0]
9
monocytes 0.2 x 10 /L [0.2-1.2]
Which one of the following treatments will result in the most rapid rise in the platelet count?
A. Anti-D immunoglobulin.
B. Danazol.
C. Dexamethasone.
D. Intravenous immunoglobulin.
E. Prednisolone.
Question 33
A 15-month-old boy presents with poor weight gain, lethargy, irritability and pallor. He has had five to six loose
sloppy bowel motions a day for the last three weeks. Prior to this he was well. He is referred for a small bowel
biopsy, which shows partial villous atrophy.
What is the most likely organism to have caused this clinical picture?
A. Campylobacter jejuni.
B. Enterotoxigenic E. coli.
C. Rotavirus.
D. Salmonella typhimurium.
E. Vibrio cholerae.

19 P299
Question 34
A seven-year-old boy wakes one morning with severe leg pain, predominantly in the calf muscles after an upper
respiratory tract infection four days previously. He is unable to walk but is otherwise well. The casualty officer
thinks that there is weakness distally and has difficulty obtaining reflexes. The boy experiences a lot of calf pain on
examination. His serum creatine kinase is 2,000 U/L [40-240].
A. Dermatomyositis.
B. Guillain-Barré syndrome.
C. Reactive arthritis.
D. Rhabdomyolysis.
E. Viral myositis.
Question 35
A 17-year-old boy, repeating the second last year of secondary schooling, has been previously diagnosed by a
paediatrician and a psychologist, as suffering from Attention Deficit Hyperactivity Disorder (ADHD) and a specific
learning disorder. Central nervous system stimulants were helpful but discontinued because of poor sleep and
volatile mood. The paediatrician is overseas and the boy now comes to you for help because he cannot study, is
failing at school and he wants to go back on dexamphetamine.
He comes from a well functioning family in which there is no history of psychiatric illness or developmental
disorder. His parents report a progressive decline in his academic progress throughout secondary school and his
network of friends has been steadily reducing. He complains that his parents and teachers are too critical of him
but admits that he often cannot be bothered studying and uses alcohol and marijuana but denies other drug use.
He says that he has not slept and has eaten little over the last few days. He worries that people are talking about
him.
He looks unkempt, is agitated, restless and guarded. His affect is flat and you have trouble following his thought
processes. He denies that he is hearing voices.
What is the most likely cause of his latest presentation?
A. Attention Deficit Hyperactivity Disorder.
B. Bi-polar disorder (manic depressive psychosis).
C. Chronic marijuana abuse.
D. Depression.
E. Schizophrenia.

20 P299
Question 36
A five-year-old boy presents with a history of macroscopic haematuria. Urine microscopy demonstrates 3,000 red
blood cells per microlitre which are dysmorphic.
The single most useful diagnostic investigation is:
A. coagulation profile.
B. renal ultrasound.
C. serum complement (C3, C4).
D. urine calcium/creatinine ratio.
E. urine culture.
Question 37
A 22-month-old girl presents with a hoarse voice, intermittent stridor, marked recession and fever. She has had
four to five such episodes over the past six months but her voice has always been hoarse over this period.
A. bronchogenic cyst.
B. infantile larynx.
C. laryngeal papillomatosis.
D. lingual cyst.
E. vascular ring.
Question 38
A nine-month-old boy, who has been fully immunised, presents with a pneumonia and has the following results:
IgG 1.1 g/L [2.1-12.2]

IgA <0.10 g/L [0.17-1.20]
IgM 0.15 g/L [0.32-1.40]
IgE <5 kU/L [0-35]
Lymphocyte markers:
CD3 (T cells) 93% [53-71]
CD4 (T helper) 68% [28-52]
CD8 (T suppressor) 24% [13-31]
CD19 (B cells) 0% [19-38]
natural killer (NK) cells 4% [3-12]
His pneumonia is most likely to be due to which one of the following organisms?
A. Escherichia coli.
B. Haemophilus influenzae type b.
C. Mycoplasma pneumoniae.
D. Pneumocystis carinii.
E. Staphylococcus aureus.

21 P299
Question 39
A two-year-old boy presents with a 10-month history of loose stool and a 10-day history of swelling of both feet.
On examination he is a pale looking child with bilateral oedema of the feet and ankles. There were no rashes or
lymphadenopathy. He has a 2/6 ejection systolic murmur at the left sternal edge. The respiratory and abdominal
examinations were normal. The following investigations were performed:

urea 2.5 mmol/L [1.3-6.6]
alanine aminotransferase (ALT) 20 U/L [10-45]
gamma glutamyltransferase (GGT) 11 U/L [<40]
bilirubin (total) 3 µmol/L [<10]
total protein 45 g/L [57-80]
urinalysis pH 7.2 negative for protein and blood
3
urine culture 10 /mL mixed growth
Stool microscopy: no red blood cells, white blood cells or mucus

no bacteria or parasites seen
Stool culture: no bacterial pathogens identified
What is the most appropriate test to perform?
A. Alpha-1-antitrypsin clearance study.
B. Barium meal and follow through.
C. Echocardiogram.
D. Renal ultrasound.
E. 24-hour urinary protein excretion.
Question 40
A two and a half-year-old girl is referred for developmental assessment. Her parents report that she has 10 to 15
single words in her vocabulary and one recognisable two-word phrase. Her pronunciation of words is not always
clear. She seems to understand most things said to her. Audiological testing is normal. She is physically very
active and finds it difficult to settle to task. However, she can sit and watch television for up to five minutes. She
plays with toy cars by pushing them up and down repeatedly and making engine noises. She also enjoys playing
with dolls and will kiss, hug, scold, pretend to feed them, and push them around in a toy pram. However, she does
not play cooperatively or interactively with other children, and is somewhat self-absorbed. She runs, climbs a
playground slide, scribbles with a crayon and can feed herself with a spoon. She can also drink from a cup and
take off some of her clothes. She tantrums if things do not go her way and screams on separation from her
mother. She becomes highly agitated and cries when the vacuum cleaner is turned on. She is also frightened of
the neighbour's dog.
What is the one best explanation for this child?
A. Anxiety disorder.
B. Autistic spectrum disorder.
C. Intellectual disability.
D. Isolated speech delay.
E. Normal variation.

22 P299
Question 41
A two-year-old boy, previously well, presents with a four-day history of cough, fever and lethargy. He is still eating
and drinking, although his appetite is reduced. There is no personal or family history of atopy. He has been fully
immunised, including four doses of conjugate Hib vaccine.
He is febrile to 38.5°C but looks well and is not cyanosed. His respiratory rate is 45/minute and there are signs of
crackles and bronchial breathing consistent with pneumonia. His pulse oximeter reading shows 98% saturation in
air.
The chest X-ray that was obtained is shown below.
What would be the most appropriate choice of antibiotics?
A. Intravenous cefotaxime.
B. Intravenous flucloxacillin and cefotaxime.
C. Intravenous penicillin.
D. Oral erythromycin.
E. Oral roxithromycin.
Question 42
A four-year-old boy presents with developmental delay and dysmorphic features. He is known to have a small
ventricular septal defect. The mother is 10 weeks pregnant. The parents are anxious to know if further children
could be affected by the same condition. His speech is poorly articulated and difficult to understand. He has
prominent ears, a high arched palate and a flat mid-face. The mother is described as a slow learner and has a
repaired cleft palate. A Fluorescent in-situ Hybridisation (FISH) study performed on the child using a probe from
the Di George critical region at locus 22q11 shows a deletion.
To assist in counselling these parents, the next most appropriate step is to:
A. arrange a fetal echocardiogram to be done at 24 weeks gestation.
B. arrange a FISH study on the boy using a probe specific for the Elastin gene at 7q11.23.
C. arrange a urinary metabolic screen on the child.
D. arrange for Fragile X cytogenetic studies on the child and his mother.
E. arrange for the parents to have a FISH study of locus 22q11.

23 P299
Question 43
A four-year-old boy presents with a one-month history of easy bruising. Two weeks ago he was found to be
9
thrombocytopenic (platelet count 20 x 10 /L) and was treated with prednisolone. He has six café au lait spots, and
has a repaired hypospadias. He has an asymptomatic ventricular septal defect.
His blood count is repeated:

9
(normal differential)
9
A. Acute myeloid leukaemia.
B. Bernard-Soulier disease.
C. Chronic idiopathic thrombocytopenic purpura.
D. Fanconi anaemia.
E. Wiskott-Aldrich syndrome.
Question 44
A five-day-old infant presents with focal motor seizures. A computerised tomography (CT) scan of the head was
performed and is shown below.
A. arteriovenous malformation.
B. cortical atrophy.
C. left middle cerebral artery infarct.
D. left subdural effusion.
E. subarachnoid haemorrhage.

24 P299
Question 45
A 14-year-old girl from interstate is seen in the Emergency Department with a fever. She has right thoracotomy
and median sternotomy scars and her mother reports that she had heart operations at three and seven years of
age. The chest X-ray obtained is shown below.
Her cardiac condition was most likely to have been:
A. persistent truncus arteriosus.
B. pulmonary atresia with intact ventricular septum.
C. tetralogy of Fallot.
D. transposition, ventricular septal defect and pulmonary stenosis.
E. tricuspid atresia.

25 P299
Question 46
A 16-year-old post pubertal boy has chronic renal failure with a serum creatinine of 300 µmol/L due to
glomerulonephritis. Two years ago his creatinine was 100 µmol/L and one year ago it was 200 µmol/L. He wants
to complete his schooling in two years time before undergoing pre-emptive living related transplantation. He is
growing on the 25th percentile for height and the 10th for weight (having gained height but not weight in the last
year). His blood pressure is 140/105 mmHg and he has 2.0 g per day of proteinuria. His dietary protein content is
2 g/kg/day.
His serum chemistry is:

urea 45 mmol/L [<6]
calcium 2.4 mmol/L [2.26-2.56]
phosphate 2.6 mmol/L [0.94-1.37]
parathyroid hormone 70 pg/ml [2-7]
Which one of the following will have no effect on the progression to end-stage renal failure?
A. Angiotensin converting enzyme inhibition.
B. Correction of anaemia using erythropoietin.
C. Correction of hyperphosphataemia.
D. Energy replete, protein restricted diet.
E. Reducing blood pressure.
Question 47
A 16-year-old presents with haemoptysis and consolidation of the right upper lobe and left lower lobe. His renal
function is abnormal with elevated creatinine and proteinuria. He has had bloody rhinorrhea over a three-week
period. His cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA) test is positive.
A. Churg Strauss syndrome.
B. Henoch Schonlein disease.
C. idiopathic pulmonary haemosiderosis.
D. polyarteritis nodosa.
E. Wegener's granulomatosis.

26 P299
Question 48
Hypothalamic damage as a consequence of local tumours is not typically associated with which one of the
following?
A. Absent thirst.
B. Excessive weight gain.
C. Memory disturbance.
D. Polydipsia.
E. Tremor.
Question 49
A five-week-old boy presents with poor weight gain and lethargy. He was born at term after a normal pregnancy
and delivery weighing 3100 g. Due to difficulty with breastfeeding he was commenced on a cow’s milk protein
formula on day three. He passed meconium within the first 24 hours of life, but his second stool was not until
seven days later. At seven days he was changed to a soy protein formula and since then has had a daily watery
stool.
On clinical examination he is a pale, thin infant. His abdomen is distended with palpable bowel loops but no
masses nor hepatosplenomegaly. Bowel sounds are frequent and high pitched. The stools are positive for occult
blood. An X-ray of the infant's abdomen is shown below.

27 P299
Blood investigations reveal:

9
9
urea 6.9 mmol/L [1-6]
gamma glutamyltransferase (GGT) 20 U/L [6-52]
What is the most likely diagnosis?
A. Cow’s milk protein intolerance.
B. Cystic fibrosis.
C. Hirschsprung's disease.
D. Intussusception.
E. Malrotation.
Question 50

28 P299
A 14-year-old boy presents to his general practitioner after 36 hours of a febrile upper respiratory tract infection.
Blood pressure is normal. Urinalysis shows heavy microscopic haematuria and 2+ protein. His serum creatinine
is normal. His renal biopsy is shown (light microscopy on top, electron microscopy underneath) (see previous
page).
A. focal segmental glomerulosclerosis.
B. mesangiocapillary glomerulonephritis (Type 1).
C. minimal change disease.
D. post-infectious glomerulonephritis.
E. thin glomerular basement membrane disease.
Question 51
A two-year-old boy, previously well and fully immunised, presents with three days of fever, cough and increasing
tachypnoea. His chest X-ray is shown below.
In addition to intravenous antibiotics what would be the most appropriate management plan?
A. Aspirate to dryness and repeat chest X-ray in 24 to 48 hours.
B. Diagnostic tap of 5-10 ml and base choice of antibiotics on Gram stain.
C. Insert wide bore chest drain.
D. Instil streptokinase.
E. No other treatment.

29 P299
Question 52
Prophylactic surfactant (given within 15 minutes of birth) has been compared to rescue surfactant (given
immediately after intubation for established hyaline membrane disease), in several controlled trials.
The use of prophylactic surfactant as opposed to rescue surfactant in very low birthweight infants has been shown
to decrease the incidence of which one of the following?
A. Air-leak syndrome.
B. Bronchopulmonary dysplasia.
C. Intraventricular haemorrhage (grade three to four).
D. Patent ductus arteriosus.
E. Periventricular leukomalacia.
Question 53
A four and a half-year-old girl is referred for investigation of short stature. Her birthweight was 2650 g and length
48 cm at term. Her mid parental height is 164 cm (50th percentile). At age two, her length was on the 10th
percentile. Her health is good except for recurrent otitis media.
Physical examination reveals no dysmorphic features but she has thickened tympanic membranes with fluid
behind the drums. Her current height is 95 cm (1st percentile) and weight is 14 kg (10th percentile).
Which one of the following investigations is most likely to establish a diagnosis?
A. Bone age.
B. Endomysial antibody.
C. Insulin-like growth factor 1.
D. Karyotype.
E. Thyroid-stimulating hormone.
Question 54
A 10 month old, exclusively breast fed boy is failing to thrive and has regressed in social and motor skills from age
five months. Examination shows apathy, pallor, hypotonia with hyperreflexia, inability to sit unaided and unusual
movements.
The following investigations are performed:

blood film macrocytosis
serum B12 50 ng/L [200-760]
serum folate 8 µ/L [3.1-7.1]
urine metabolic screen increased methylmalonic acid and homocystine

30 P299
The most appropriate next step would be:
A. bone marrow biopsy.
B. enzyme assay in cultured fibroblasts.
C. maternal serum B12 level.
D. Schilling test.
E. small bowel biopsy.
Question 55
Infection with which one of the following organisms causes most admissions of children to hospital in Australia and
New Zealand?
A. Bordetella pertussis.
B. Influenza viruses.
C. Parainfluenza viruses.
D. Respiratory syncytial virus.
E. Rotavirus.
Question 56
A 10-year-old girl is referred for evaluation of an intermittent fast heart beat occurring when watching television and
after retiring. Her physical examination, chest X-ray, 12 lead ECG and echocardiograph are normal. The two
illustrated tracings (see following page) are recorded from a simultaneous three channel Holter monitor system.
You would recommend treatment with which one of the following?
A. Amiodarone.
B. Flecainide.
C. Propranolol.
D. Sotalol.
E. No drugs.

31 P299

32 P299
Question 57
A nine-year-old boy presents with a long history of mild to moderate hyperactivity, impulsiveness, poor
concentration and inattentiveness. Over the last 18 months, he has developed a pattern of facial grimaces,
blinking of his eyes and snorting. He is said to be bright but is under-performing at school, especially in reading
and mathematics. He also has a history of strange behaviour in that he will not allow anyone in his family to sit at
the dinner table before him and he insists that they touch his soft toy (a dinosaur) and say hello or goodbye to the
toy when he enters or leaves the room. He has a history of mood swings and aggressive behaviour/tantrums
when thwarted, in that he will lash out at his siblings or his peers.
Which one of the following is most likely to exacerbate his abnormal movements?
A. Clonidine.
B. Desipramine.
C. Haloperidol.
D. Methylphenidate.
E. Pimozide.
Question 58
A 16-year-old male student anxiously presents to his doctor after his girlfriend noticed he was becoming yellow.
He felt well and there were no abnormalities on physical examination. He denied drug or excessive alcohol use.
He was a keen football player and had recently commenced a rigorous diet to prepare for the coming football
season.
Blood investigations revealed:
bilirubin -total 48 µmol/L [<10]

-conjugated 7 µmol/L
gamma glutamyltransferase (GGT) 13 U/L [<45]
alkaline phosphate 92 U/L [15-125]

9
white cell count 7.2 x 10 /L [4.5-13]
9
film normal
reticulocyte count 1% [<2%]
What investigation is appropriate?
A. Epstein-Barr virus serology.
B. Glucose-6-phosphate dehydrogenase screen.
C. Hepatitis B serology.
D. Liver ultrasound.
E. No investigation.

33 P299
Question 59
A non-consanguineous couple has a son who is born with craniosynostosis, cleft palate and total (glove)
syndactyly involving all limbs. No one else in the family is similarly affected. His photograph is shown below. The
mother is 35 years old and the father is 46 years old.
The risk that their next child will be affected is closest to:
A. 100%.
B. 75%.
C. 50%.
D. 25%.
E. 1%.
Question 60
A one-year-old boy presents with vomiting, fever and lethargy. His abdomen is soft, kidneys are not palpable and
6 8
his genitalia are normal. A suprapubic urine specimen contains 100 x 10 white blood cells/L and grows >10 E.
coli/L in pure growth. He responds rapidly to intravenous ampicillin and gentamicin.
What investigations, if any, are indicated?
A. DMSA scan alone.
B. Micturating cystourethrogram and renal ultrasound.
C. No investigations unless he has a second infection.
D. Renal ultrasound scan alone.
E. Renal ultrasound and DTPA scan.

34 P299
Question 61
The following results are obtained from a 14-year-old boy, prior to elective hernia surgery, who gives a history of
excessive bleeding after dental extraction.
Bleeding time 6 minutes [2-9]

Prothrombin time 12 seconds [12-14]
Prothrombin time-international normalised ratio (PT-INR) 1.0 [0.9-1.2]
Activated partial thromboplastin time (APTT) 56 seconds [26-35]
Fibrinogen 2.4 g/L [1.8-4.0]
Mixing the patient's plasma with an equal volume of normal plasma normalises the APTT.
A. Antiphospholipid antibody syndrome.
B. Factor VII deficiency.
C. Haemophilia.
D. Recent aspirin ingestion.
E. Von Willebrand disease.
Question 62
An 11-year-old girl is referred because of recurrent severe asthma and very high doses of inhaled steroids and
repeated courses of oral steroids (eight months on oral steroids in the last 12 months). She complains of severe
shortness of breath with minimal exercise. She has gained 7 kg in the past 12 months. She has missed four
months of school in the past year because of respiratory illness. Her lung function tests are normal. During an
exercise test which she terminated at six minutes of bicycling, she was noted to have ‘severe distress’ associated
with stridor and inspiratory and expiratory wheezes. Her flow volume loops (tidal volumes) during exercise are
shown above and exhibit a saw-tooth appearance.

35 P299
A. exercise induced asthma.
B. obliterative bronchiolitis.
C. psychogenic asthma.
D. recurrent spasmodic croup.
E. unstable asthma.
Question 63
A four-month-old girl is referred with tachypnoea, failure to thrive, a murmur and hepatic enlargement. This still
frame from her echocardiogram, shown below, is an apical four chamber projection displayed in the anatomical
position.
It indicates that the most likely cause of her heart failure is:
A. complete atrioventricular septal defect.
B. inlet muscular ventricular septal defect.
C. multiple ventricular septal defects.
D. primum atrial septal defect.
E. secundum atrial septal defect.

36 P299
Question 64
A 26-year-old woman with a history of recurrent genital herpes presents at 38 weeks gestation in labour. She is
found to have a small, active herpetic lesion on her cervix uteri. A caesarean section is performed four hours after
rupture of membranes. A healthy baby girl is born weighing 3600 g and examination of the baby is normal.
What would be the most appropriate management plan for this baby?
A. Commence oral acyclovir immediately.
B. Commence intravenous acyclovir immediately.
C. Take viral swabs from the baby's conjunctiva and nasopharynx immediately.
D. Take viral swabs from the baby's conjunctiva and nasopharynx 24-48 hours after delivery.
E. No action necessary, unless lesions develop.
Question 65
A 14-year-old boy in the second year of secondary schooling has always been anxious, insecure and isolated but
has had no previous panic or phobic symptoms. He has a few friends, but at times he behaves in an inappropriate
manner. At school, he is an average student. His teachers believe he is immature but otherwise normal.
For the last two years, he has been masturbating, preoccupied with sexual matters and has interfered with his
parents' conversations with other adults. He worries that whenever his parents go out, they are having affairs with
other people. Over the last four months he has become irritable, moody and angrily reacts to even mild criticism
from his older brothers. He has developed a fear of germs and of being contaminated. After he has emptied his
bowels he has to have a shower and he worries about stepping on dirty band-aids. He also worries about being
attacked when he rides his bike and has to repeatedly check his windows at night, to ensure that they are locked
but even so, he finds it difficult to sleep. He spends large amounts of time with his mother but worries that if she
touches his clothes, she may become pregnant. He mostly believes that these fears are "silly" but cannot stop
worrying.
A. Anxiety disorder.
B. Depression.
C. Obsessive-compulsive disorder.
D. Phobic disorder.
E. Schizophrenia.

37 P299
Question 66
The following investigations are performed on a 12-week term infant.
IgG 3.08 g/L [1.45-11.28]

IgA <0.10 g/L [0-0.55]
IgM 0.74 g/L [0.21-0.88]
IgE 5 KU/L [0-45]
Whole blood PHA response 49% [70-150]
CD3 42% [53-75]
CD4 8% [23-60]
CD8 34% [14-25]
CD19 23% [12-39]
Natural killer cells 15% [3-9]
9
White cell count 17.7 x 10 /L [5.0-19.5]
12
Red cell count 4.6 x 10 /L [2.7-4.9]
Haemoglobin 116 g/L [90-140]
9
Platelet count 238 x 10 /L [150-400]
Mean corpuscular volume 77 fL [77-115]
Mean corpuscular haemoglobin 26 pg [26-34]
MANUAL DIFFERENTIAL
9
Band forms 0.18 x 10 /L (1%)
9
Neutrophils 11.86 x 10 /L (67%) [1.0-9.0]
9
Lymphocytes 4.78 x 10 /L (27%) [2.5-16.5]
9
Monocytes 0.89 x 10 /L (5%) [0.2-1.5]
These results are most consistent with:
A. Di George syndrome.
B. HIV infection.
C. isolated IgA deficiency.
D. severe combined immunodeficiency.
E. Wiskott-Aldrich syndrome.
Question 67
A three-year-old girl develops otitis media and is treated with an antimicrobial agent. Eight days later she presents
with fever, an urticarial rash and painful swelling of her ankles and right knee. Examination shows a resolving otitis
media and confirms there is some swelling and painful limitation of movement of the ankles and right knee. An
urticarial rash is noted. She has a temperature of 38.5°C.
Which one of the following antimicrobial agents is most likely to have caused this clinical picture?
A. Amoxycillin.
B. Amoxycillin-clavulanic acid.
C. Cefaclor.
D. Cotrimoxazole.
E. Erythromycin.

38 P299
Question 68
A 10-month-old girl presents with a one-month history of refusal to weight bear on her left leg. She also complains
when her nappies are changed. There is no history of trauma nor fevers.
The radiograph below was taken.
A. Chronic osteomyelitis.
B. Congenital cyst.
C. Developmental dysplasia of the hip.
D. Non-accidental injury.
E. Osteoid osteoma.
Question 69
A 12-year-old girl is referred with a provisional diagnosis of absence epilepsy. On specific questioning she
expresses concern that she often shakes and drops objects, particularly in the mornings. There is no family
history of epilepsy. Neurological examination is normal. School progress is satisfactory. An EEG reveals 4-6 Hz
bilaterally symmetrical polyspike and wave patterns with normal background.
A. Benign occipital epilepsy.
B. Benign partial epilepsy of childhood with Rolandic spikes.
C. Childhood absence epilepsy.
D. Complex partial epilepsy.
E. Juvenile myoclonic epilepsy.

39 P299
Question 70
A 10-year-old boy growing on the 50th percentile for height and weight has oliguric end stage renal failure treated
with continuous ambulatory peritoneal dialysis. He has four exchanges of one litre of 1.5% dialysate per day. He
presents for review and is found to have gained 7 kg in weight over the last month. His jugular venous pulse
measured 5 cm and his resting respiratory rate was 40/minute.
The least appropriate treatment would be:
A. administering a high dose of frusemide.
B. decreasing his daily fluid intake.
C. increasing the frequency of dialysate bag change.
D. increasing the strength of dialysate.
E. increasing the volume of dialysate used for dialysis.
Question 71
After an apparently normal discharge examination on day three, a five-day-old infant is brought to the Emergency
Department because of severe shortness of breath and feeding difficulties. On examination she is markedly
tachypnoeic, cyanosed and mottled. It is not possible to feel any pulses and both blood pressure and pulse
oximetry are not recordable. An ECG monitor shows her heart rate to be 180/minute. She has a prominent
parasternal heave and a liver edge 6 cm below the costal margin. On auscultation she has a gallop rhythm but no
obvious murmurs. Her chest X-ray is shown below.
A. aortic valve atresia.
B. atrioventricular septal defect.
C. tetralogy of Fallot.
D. transposition with ventricular septal defect.
E. truncus arteriosus.

40 P299
Question 72
A four-year-old boy with short bowel syndrome due to necrotising enterocolitis presents with recurrent episodes of
abdominal distension, vomiting and loose stools. Since ceasing parenteral nutrition two years ago he has received
continuous gastrostomy feeds with Presgestemil and eats a limited diet. He is gaining weight and growing just
below the 3rd percentile for age. On examination he is lethargic but not unwell. He is not dehydrated. His
abdomen is distended with no masses and 2 cm of splenomegaly. Bowel sounds are increased but normal in
character. His plain abdominal X-ray is shown below.
The most appropriate next step would be:
A. abdominal ultrasound.
B. barium meal and follow through.
C. breath hydrogen test.
D. exploratory laparotomy.
E. gastroduodenal manometry.

41 P299
Question 73
A four and a half-year-old girl is seen for review of her diabetes. She was diagnosed nine months previously and
her parents report no particular problems. Her weight is 17 kg, her insulin is 3 U Actrapid / 7 U Protophane prior to
breakfast and 1 U Actrapid / 4 U Protophane prior to tea. Her blood sugar level when seen is 4.4 mmol/L and her
Hb A, C level is 6.8% [non diabetic range: 4.5-6].
The last seven days' levels have been accurately recorded.
Morning Lunch Tea Supper
5.0 6.3 11.0 4.0

3.5 7.4 5.6 4.8
4.8 3.5 7.1 3.6
3.6 3.8 4.5 6.1
7.4 6.5 3.8 5.6
3.1 6.0 4.0 3.5
4.1 4.8 12.2 10.8
Which one of the following recommendations would you make?
A. Change from human to beef insulin.
B. Increase carbohydrate at breakfast and supper.
C. Continue current dose.
D. Reduce insulin dose.
E. Reduce the number of blood sugar levels recorded.
Question 74
The following information was obtained at cardiac catheterisation in a six-month-old infant with a loud systolic
murmur at the lower left sternal edge:
Saturation (%) Pressure (mmHg)

inferior vena cava 70
superior vena cava 69
right atrium 69 mean = 7
right ventricle 88 40/7 [25/5]
main pulmonary artery 89 40/24 [25/12]
left atrium 99 mean = 8
left ventricle 99 85/7 [90/5]
aorta 99 85/50 [90/60]
Which one of the following additional auscultatory murmurs is most likely to be heard?
A. A continuous murmur at the upper left sternal edge.
B. A mid diastolic murmur at the apex.
C. A mid diastolic murmur at the lower left sternal edge.
D. An early diastolic murmur at the upper left sternal edge.
E. An ejection systolic murmur at the upper left sternal edge.

42 P299
Question 75
A nine-month-old boy is seen in the Emergency Department following a one minute generalised seizure. He has
normal development and clinically is afebrile and has no focal neurological signs. His height and weight are on the
10th percentile. His parents are non-consanguineous Lebanese. The following investigations are performed:
urea 3 mmol/L [1-6]

bicarbonate 22 mmol/L [22-27]
calcium 1.45 mmol/L [2.1-2.6]
phosphorus 1.2 mmol/L [1.2-1.8]
serum alkaline phosphatase 600 U/L [80-320]
parathyroid hormone 0.8 ng/ml [<0.5]
A. Hypophosphatemic rickets.
B. Pseudohypoparathyroidism.
C. Renal tubular acidosis.
D. Vitamin D deficient rickets.
E. Vitamin D resistant rickets.
Question 76
You have been asked to review the biochemical profile of a 13-year-old girl who is undergoing nasogastric feeding
for severe anorexia nervosa, which was initiated three days earlier. You calculate that she is receiving 100
calories per hour. She had normal biochemistry on admission.
Her current biochemical profile is:

urea 1.0 mmol/L [2.1-6.5]
glucose 2.4 mmol/L [3.5-5.4]
calcium 1.99 mmol/L [2.10-2.60]
phosphate 0.8 mmol/L [1.1-1.8]
This picture is most likely to be due to which one of the following?
A. Addisonian crisis.
B. Diuretic abuse.
C. Laxative abuse.
D. Secondary renal tubular acidosis.
E. The enteral nutrition.

43 P299
Question 77
A six-month-old baby, exclusively breastfed, presents with failure to thrive and intermittent fevers of one month's
duration.
What does the baby's chest X-ray reveal?
A. Azygous lobe.
B. Mediastinal tumour.
C. Lymphoma.
D. Normal thymus.
E. Right upper lobe pneumonia.
Question 78
Which one of the following conditions is most likely to be responsive to treatment with interferon alpha?
A. Arterio-venous malformation.
B. Hepatoblastoma.
C. Large capillary haemangioma.
D. Neuroblastoma.
E. Wilms tumour.

44 P299
Question 79
In Kawasaki disease, which one of the following laboratory findings would be the least likely?
A. Aseptic meningitis.
B. Elevated serum transaminases.
C. Sterile pyuria.
D. Thrombocytopenia.
E. Toxic granulation of neutrophils.
Question 80
An 11-week-old infant presents with irritability, poor weight gain and jaundice. She was born weighing 3300 g after
a normal pregnancy and delivery. She was the first child of non-consanguineous parents. She was exclusively
breastfed and grew satisfactorily until eight weeks of age. At 11 weeks of age she was referred because of poor
weight gain and jaundice. On examination she is lethargic, pale and icteric with no rashes, bruising or
lymphadenopathy. Her length has decreased from the 50th to 30th percentile and weight from the 50th to 15th
percentile in the past month. Her head circumference chart is shown below. She has 3 cm of firm splenomegaly
and 1 cm of hepatomegaly. There is no ascites. The stool is a normal yellow green colour.

45 P299
Blood tests show the following:

9
white cell count 15.3 x 10 /L [4.5-13.5]
9
Film: moderate numbers of spherocytes and fragmented cells.
bilirubin - total 143 µmol/L [<30]

- conjugated 121 µmol/L [0-40]
alanine aminotransferase 120 U/L [10-50]
gamma glutamyltransferase 210 U/L [0-40]
prothrombin time 26 seconds [10-13]
activated partial thromboplastin time 85 seconds [25-38]
Abdominal ultrasound: liver and spleen enlarged with normal echogenic appearance.
No dilation of intra or extrahepatic ducts. No ascites.
A. Alagille syndrome.
B. cystic fibrosis.
C. extrahepatic biliary atresia.
D. metabolic liver disease.
E. neonatal hepatitis.
Question 81
A five-year-old boy is seen with a history of recurrent wheezing episodes. His parents have managed these
episodes by intermittent use of nebulised bronchodilators and when away from home have used his mother's
"Ventolin" inhaler. The parents have agreed to a trial of preventative medication.
The most appropriate method of administration would be:
A. by aerosol alone with careful parental supervision.
B. by use of aerosol via a large volume spacer.
C. by use of aerosol via a small volume spacer with mask.
D. by use of dry powder inhalation.
E. by use of the nebuliser.

46 P299
Question 82
Patients with Marfan syndrome and homocystinuria have a number of similar clinical features.
In a given patient, which one of the following is most likely to suggest homocystinuria rather than Marfan
syndrome?
A. Autosomal dominant inheritance.
B. Ectopia lentis.
C. Intellectual impairment.
D. The absence of a history of arterial thrombosis.
E. The presence of abnormally long legs and arms.
Question 83
In an asymptomatic person with human immunodeficiency virus (HIV) infection, which one of the following is the
best predictor of the future rate of decline of immune function?
+
A. CD4 lymphocyte count.
+
B. CD8 lymphocyte count.
C. p24 antigenaemia.
D. Plasma HIV RNA concentration.
E. Serum β2 microglobulin concentration.
Question 84
A three-year-old girl presents with an abnormal gait but no other symptoms. Her photograph is shown below.
Apart from the abnormality shown, her examination is otherwise normal.

47 P299
Which one of the following is most commonly associated with her abnormality?
A. Craniopharyngioma.
B. Ewing sarcoma.
C. Osteosarcoma.
D. Retinoblastoma.
E. Wilms tumour.
Question 85
A 15-year-old boy presents to his paediatrician with short stature and headaches. Magnetic resonance imaging of
his brain is shown below.
A. craniopharyngioma.
B. eosinophilic granuloma.
C. hypothalamic hamartoma.
D. pinealoma.
E. pituitary adenoma.

48 P299
Question 86
A 13-year-old boy with severe spastic quadriplegia is referred for consideration of placement of a gastrostomy tube
to aid with feeding. He has scoliosis and chronic lung disease.
Which one of the following factors would be a contraindication to percutaneous endoscopic gastrostomy tube
placement in this patient?
A. Oropharyngeal incoordination.
B. Past history of appendicectomy.
C. Recurrent constipation.
D. Severe gastro-oesophageal reflux.
E. Severe generalised hypertonia.
Question 87
Which anti-arrhythmic drug is inappropriate in the treatment of the arrhythmia with which it is paired?
A. Atrial flutter: sotalol.
B. Atrial tachycardia in Wolff-Parkinson-White syndrome:flecainide.
C. Prolonged QT syndromes: propranolol.
D. Sinus node dysfunction: digoxin.
E. Ventricular tachycardia in cardiomyopathy: amiodarone.
Question 88
A 16-year-old girl has lost 13 kg over the past eight months. For the past four months she has had amenorrhoea.
On examination you note that she is thin but alert and active. There is a yellowish discolouration of her skin. You
suspect anorexia nervosa.
In the patient's medical history, the one feature least consistent with the diagnosis of anorexia nervosa would be:
A. concern about being too thin.
B. excessive exercise.
C. laxative abuse.
D. preoccupation with food.
E. ritualistic eating behaviours.

49 P299
Question 89
A five-year-old girl with a two-year history of steroid responsive nephrotic syndrome has experienced four relapses
over this period. Three months ago she relapsed and was treated with oral prednisolone in a dose of 1 mg/kg/day.
Her oedema resolved but her proteinuria on albustix testing persisted at 2+. The prednisolone dose has been
tapered to 0.5 mg/kg on alternate days. She now presents with generalised oedema and gross ascites with 4+
proteinuria. A renal biopsy was performed.
The picture above shows a representative glomerulus (stained with PAS and silver). Immunofluorescent staining
for immune deposits was negative.
Which one of the following is the most appropriate therapeutic response?
A. An angiotensin converting enzyme inhibitor.
B. Azathioprine.
C. Cyclophosphamide.
D. Cyclosporin.
E. Prednisolone 2 mg/kg/day.

50 P299
Question 90
A three-year-old girl with Alagille's syndrome has an X-ray taken, which is shown below.
What is the most likely cause for the changes demonstrated?
A. Delayed bone mineralisation secondary to chronic disease.
B. Magnesium deficiency.
C. Rifampicin toxicity.
D. Vitamin C deficiency.
E. Vitamin D deficiency.
Question 91
The most common presenting feature of retinoblastoma is:
A. decreased visual acuity.
B. ocular inflammation.
C. proptosis.
D. strabismus.
E. white reflex.

51 P299
Question 92
A three-month-old infant is recognised as having an early systolic murmur, grade 3/6 in intensity at the lower left
sternal edge and towards the apex. It starts abruptly with the first heart sound and disappears before the second.
Examination is otherwise normal.
A. atrial septal defect.
B. tetralogy of Fallot.
C. peripheral pulmonary artery stenosis.
D. pulmonary valve stenosis.
E. small muscular ventricular septal defect.
Question 93
A 14-year-old girl presents to her paediatrician with early morning headache and vomiting. She was later
diagnosed with benign (idiopathic) intracranial hypertension.
Which one of the following has not been associated with benign intracranial hypertension?
A. Nalidixic acid.
B. Nitrofurantoin.
C. Penicillin.
D. Prednisolone.
E. Tetracylines.
Question 94
This 13-year-old girl with spina bifida (see following page) presents with acute urticaria and hypotension during
surgery for appendicitis. She was resuscitated with plasma expanders. Exposure to the following products
occurred.
Which one is most likely to have caused the reaction?
A. Crystalloid.
B. Gentamicin.
C. Halothane.
D. Latex products.
E. Succinyl choline.

52 P299
Question 95
A 12-year-old girl presents with chronic diarrhoea for four months with associated abdominal pain and tenesmus.
There is a past history of constipation. Her appetite and energy level have decreased but there has been no
weight loss. Abdominal examination reveals a firm non-tender mass in the right iliac fossa and suprapubic region.
Stool microscopy and culture have been negative. Her X-ray is shown (see following page).
A. chronic constipation.
B. inflammatory bowel disease.
C. intestinal lymphoma.
D. ovarian tumour.
E. rhabdomyosarcoma of the bladder.

53 P299
Question 96
A 10-year-old boy is found to have heavy microscopic haematuria when seen by his General Practitioner after 36
hours of febrile upper respiratory tract infection. Blood pressure is normal. Urinalysis shows blood and 2+ protein.
Serum creatinine and complement C3 and C4 are normal.
A. Goodpasture's syndrome (anti-glomerular basement membrane disease).
B. IgA nephropathy.
C. lupus nephritis.
D. mesangiocapillary glomerulonephritis.
E. post-infectious glomerulonephritis.

54 P299
Question 97
A six-month-old girl presents with a 14-day history of diarrhoea. The illness initially began with fever, vomiting and
diarrhoea. Her vomiting and fever resolved after 36 hours, however, her stools have remained watery and loose.
She has recommenced on her usual cow’s milk based formula and solids including pureed fruit and vegetables.
On examination she is a tired but not unwell looking girl. Her weight is on the 25th percentile for age and height is
on the 50th percentile for age. She is not dehydrated or clinically pale. She has no rashes. Her abdominal
examination reveals a soft non-tender abdomen with no masses or hepatosplenomegaly. The following blood
tests were performed:

urea 2.5 mmol/L [1.3-6.6]
Examination of the stool is most likely to reveal:
A. pH 3.
B. reducing substance negative.
C. rotavirus antigen.
D. sodium 110 mmol/L.
E. white blood cells.
Question 98
A 10-day-old baby with Down syndrome, born after a high forceps delivery, develops poor feeding, hypothermia
and lethargy. A magnetic resonance imaging (MRI) scan with contrast of her brain was performed and is shown
below.

55 P299
The MRI is most suggestive of:
A. a cerebral abscess.
B. an infarct involving the right internal capsule.
C. an intracerebral haematoma.
D. intracerebral calcification.
E. ring artefact.
Question 99
A 10-year-old girl with common variable immunodeficiency presents with a five-day history of cough, fever and
shortness of breath.
Which one of the following organisms is most likely to be the cause of her respiratory symptoms?
A. Cytomegalovirus.
B. Mycobacterium avium complex.
C. Pneumocystis carinii.
D. Pseudomonas aeruginosa.
E. Streptococcus pneumoniae.
Question 100
A five-year-old boy presents with a three-week history of cough. Both he and his father complained of an irritating
cough, which occurs both during the day and at night. Once he starts coughing he is unable to stop for some
minutes. He has had no coryza and no fever. He recently started at primary school and has been tearful on
separating from his mother each day.
His father had mild asthma as a child. The boy's coughing bouts have not responded to inhaled salbutamol. He is
fully immunised.
On physical examination, the boy is afebrile, has no chest deformity and is not in respiratory distress. Air entry is
good bilaterally.
A chest radiograph is normal.
What is the one most likely explanation for his recent cough?
A. Adenovirus infection.
B. Asthma.
C. Mycoplasma pneumoniae.
D. Pertussis.
E. Psychogenic cough.

56 P299
1999 FRACP Written Examination
Paediatrics & Child Health
Paper 2 – Clinical Applications
Answers
1. E 34. E 67. C
2. E 35. E 68. A
3. D 36. C 69. E
4. D 37. C 70. A
5. A 38. B 71. A
6. A 39. A 72. C
7. B 40. D 73. D
8. D 41. C 74. B
9. D 42. E 75. D
10. A 43. D 76. E
11. A 44. C 77. D
12. E 45. D 78. C
13. B 46. B 79. D
14. A 47. E 80. D
15. E 48. E 81. B
16. C 49. C 82. C
17. E 50. E 83. D
18. A 51. C 84. E
19. D 52. A 85. A
20. C 53. D 86. D
21. B 54. C 87. D
22. C 55. D 88. A
23. E 56. E 89. E
24. E 57. D 90. E
25. D 58. E 91. E
26. D 59. E 92. E
27. C 60. B 93. C
28. D 61. C 94. D
29. D 62. C 95. A
30. A 63. A 96. B
31. C 64. D 97. A
32. D 65. C 98. A
33. C 66. A 99. E
100. D

1999 FRACP Written Examination Paediatrics &amp; Child

Diunggah oleh

Informasi Dokumen

Deskripsi Asli:

Hak Cipta

Format Tersedia

Bagikan dokumen Ini

Bagikan atau Tanam Dokumen

Opsi Berbagi

Apakah menurut Anda dokumen ini bermanfaat?

Apakah konten ini tidak pantas?

Hak Cipta:

Format Tersedia

1999 FRACP Written Examination Paediatrics &amp; Child

Diunggah oleh

Hak Cipta:

Format Tersedia

1999 FRACP Written Examination

Paediatrics & Child Health

Paper 2 – Clinical Applications

Paper 1 – Medical Sciences: 70 questions; time allowed: 2 hours

In the questions, values appearing within [ ] refer to normal ranges.

A table of answers is located at the end of each paper.

Copyright © 2003 by The Royal Australasian College of Physicians

The risk of the baby acquiring HIV is closest to:

Which of the following is the most likely diagnosis?

B. Congenital lobar emphysema.

The following results are obtained from a two-day-old male infant.

IgG 6.53 g/L [5.34-16.94]

Copyright © 2003 by The Royal Australasian College of Physicians

D. Severe combined immune deficiency.

haemoglobin 103 g/L [110-140]

Which one of the following is the one most likely diagnosis?

A. Coxsackie virus infection.

D. Systemic juvenile chronic arthritis.

E. Systemic lupus erythematosus.

Copyright © 2003 by The Royal Australasian College of Physicians

The most likely diagnosis is:

A. benign occipital seizures.

E. raised intracranial pressure.

Which one of the following diagnoses is most likely?

A. Congenital adrenal hyperplasia.

B. Glycogen storage disease.

E. Medium chain acyl co-A deficiency.

Copyright © 2003 by The Royal Australasian College of Physicians

B. Modified Blalock shunt (systemic to pulmonary artery anastomosis).

C. Modified Fontan procedure (total cavopulmonary connection).

D. Pulmonary artery banding.

The most likely diagnosis is:

Copyright © 2003 by The Royal Australasian College of Physicians

The risk that she is a Fragile X carrier is closest to:

A. Intravenous ampicillin and gentamicin.

D. Oral amoxycillin-clavulanic acid.

Copyright © 2003 by The Royal Australasian College of Physicians

The most appropriate initial management would be:

D. reassurance that this behaviour will settle without treatment.

E. scheduled waking of the child at 10 p.m.

bone age 15 years

The major cause of his short stature is:

A. attenuated pubertal growth spurt.

B. attenuated spinal growth.

C. familial short stature.

D. growth hormone deficiency.

Physical examination shows no specific abnormality.

Copyright © 2003 by The Royal Australasian College of Physicians

The most likely diagnosis is:

B. attention deficit hyperactivity disorder (ADHD).

C. Glutamic acid decarboxylase antibody.

E. Thyroid microsomal antibody.

Copyright © 2003 by The Royal Australasian College of Physicians

What is the most appropriate management of this baby?

A. Give intravenous acyclovir.

B. Give oral acyclovir.

C. Give intramuscular zoster immune globulin.

D. Give intramuscular zoster immune globulin and intravenous acyclovir.

haemoglobin 75 g/L [115-140]

Copyright © 2003 by The Royal Australasian College of Physicians

Peripheral blood smear shows spherocytosis as shown below.

The next most appropriate test is:

A. Coombs direct antiglobulin test.

1999 FRACP Written Examination Paediatrics & Child

1999 FRACP Written Examination Paediatrics & Child