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2001 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 P201
Question 1

A three-year-old boy presents with a two-week history of profuse, watery diarrhoea. He has a history of otitis
media for which he was treated with amoxycillin. Laboratory tests reveal the following findings:

serum sodium 138 mmol/L [135-145]


serum potassium 4.3 mmol/L [3.5-5.5]
faecal sodium 98 mmol/L
faecal potassium 32 mmol/L
stool reducing sugars 0.25%
stool pH 6.5

These findings are most consistent with:

A. coeliac disease.

B. cryptosporidiosis.

C. lactose intolerance.

D. laxative abuse.

E. sucrase-isomaltase deficiency.

Question 2

A 27-month-old boy is referred for assessment. His parents have observed approximately 10 episodes in the
past three months where he has seemed distressed and unstable on his legs. The episodes have been brief
(15-20 seconds) and recent episodes have seen him suddenly stop, close his eyes and grasp at a nearby wall
or door for the duration. He may become pale during an episode, but no other signs have been observed by
his parents and he quickly resumes his activities.

Detailed neurological examination is normal although fundoscopy is not possible. Family history includes a
maternal uncle with migraine but no family history of epilepsy. His past medical history reveals a normal
pregnancy and delivery. He had a three-minute febrile convulsion at 15 months of age in association with
otitis media. Neurodevelopmental progress is normal.

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

A. Benign paroxysmal vertigo.

B. Breath-holding episodes.

C. Cardiac arrhythmia.

D. Epilepsy.

E. Migraine.

Copyright © 2003 by The Royal Australasian College of Physicians


3 P201
Question 3

A two-month-old girl had a murmur identified at birth. To your assessment now, she is pink and thriving. She
shows no signs of respiratory distress or other signs of heart failure. The S2 is single and there is a long
systolic murmur heard loudest at the left sternal edge. Her chest X-ray is normal and her ECG is shown
above.

Which one of the following is the most likely diagnosis?

A. Aortic stenosis.

B. Atrial septal defect.

C. Physiological peripheral pulmonary stenosis.

D. Small ventricular septal defect.

E. Tetralogy of Fallot.

Copyright © 2003 by The Royal Australasian College of Physicians


4 P201
Question 4

A 14-year-old boy presents with a two-week history of fever and increasing shortness of breath. He had a
renal transplant from a cytomegalovirus (CMV)-negative donor three months previously. Auscultation of his
chest is normal. His oxygen saturation in room air is 92%. His chest X-ray is shown below. He has been
treated with cyclosporin A, prednisolone, azathioprine, nystatin, ranitidine, lisinopril and pentamidine which
was last administered by aerosol nebulisation six weeks previously.

Which one of the following treatments would be the most appropriate for this boy?

A. Aciclovir.

B. Cefotaxime.

C. Cotrimoxazole.

D. Fluconazole.

E. Penicillin.

Question 5

Which one of the following vaccines is contraindicated in a child with anaphylactic egg allergy?

A. Bacille Calmette-Guérin (BCG).

B. Influenza.

C. Measles-mumps-rubella (MMR).

D. Oral polio.

E. Whole cell pertussis.

Copyright © 2003 by The Royal Australasian College of Physicians


5 P201
Question 6

A four-year-old child, on no medications, presents with fever and is generally unwell.

Meningococcal sepsis is least likely if there is:

A. a macular, blanching rash.

B. a maculopapular rash.

C. a mixed maculopapular and petechial rash.

D. an urticarial rash.

E. no rash.

Question 7

An eight-year-old overweight girl presents with a velvety, pigmented rash on her neck and axilla (shown
below).

Which one of the following investigations is the most relevant for her future management?

A. Fasting serum cholesterol/triglyceride.

B. Fasting serum insulin/glucose ratio.

C. Plasma leptin.

D. Serum adrenocorticotrophic hormone (ACTH)/cortisol.

E. Serum thyroid-stimulating hormone (TSH).

Copyright © 2003 by The Royal Australasian College of Physicians


6 P201
Question 8

A 13-year-old boy with a history of cystic fibrosis and complex partial seizures is currently on carbamazepine.
He develops a bout of sinusitis and is prescribed an antibiotic. Two days later he presents to the Emergency
Department with a depressed conscious state and recurrent seizures. He is found to have markedly elevated
carbamazepine levels.

Which one of the following antibiotics is he most likely to have been prescribed?

A. Amoxycillin-clavulanic acid.

B. Cefaclor.

C. Ciprofloxacin.

D. Doxycycline.

E. Erythromycin.

Question 9

Successful active management of a child with nocturnal enuresis is most dependent on the child’s:

A. age.

B. bladder size.

C. family history.

D. fluid intake.

E. motivation.

Question 10

A seven-month-old girl presents with a four-week history of chronic diarrhoea and failure to thrive. She was
entirely bottle-fed with cow’s milk formula until five months of age. A range of solids, including tinned fruit and
vegetables, was then introduced into her diet. There is no history of vomiting or fevers. The diarrhoea is
watery and has caused significant perianal excoriation.

Faecal biochemical tests are likely to show which one of the following?

pH Reducing Total
sugars sugars

A. 7.2 0.25% 2%

B. 7.2 2% 2%

C. 7.2 0.25% 0.25%

D. 7.6 0.25% 0.25%

E. 7.6 0.25% 2%

Copyright © 2003 by The Royal Australasian College of Physicians


7 P201
Question 11

A 10-year-old boy presents with a 13-month history of recurrent eye-blinking and rapid movements of his
eyes, with recurrent throat clearing. The events tend to wax and wane but are most prominent at school or
when he is under stress. His paediatrician sees him and a diagnosis of Tourette syndrome is made.

Which one of the following is most likely to be identified on family history?

A. Epilepsy.

B. Essential tremor.

C. Huntington disease.

D. Obsessive-compulsive disorder.

E. Wilson disease.

Question 12

A three-week-old boy presents with a one-week history of cough. The cough is not present all the time but
comes in bouts lasting up to a minute. For two days the baby has been breathing faster and has been having
difficulty feeding. He has had no fever.

He was born by vaginal delivery at term to an 18-year-old primigravida mother. The pregnancy was normal.
He is bottle-fed. At one week of age he developed bilateral conjunctivitis which responded to
chloramphenicol eye drops.

On examination the baby is afebrile. He is in mild respiratory distress, with a respiratory rate of 52/minute,
heart rate of 140/minute and moderate intercostal recession. He is not cyanosed. He has some fine crackles
audible at both lung bases. His chest is not clinically hyperexpanded. His heart is not enlarged and heart
sounds are normal. His oxygen saturation by pulse oximetry is 94%. His chest X-ray is shown below.

Which one of the following is the most likely infecting organism?

A. Bordetella pertussis.

B. Chlamydia trachomatis.

C. Group B Streptococcus.

D. Pneumocystis carinii.

E. Respiratory syncytial virus.

Copyright © 2003 by The Royal Australasian College of Physicians


8 P201
Question 13

A 36-year-old primigravida (II:3) presents at nine weeks gestation. She requests advice about the risk of
haemophilia A for her foetus (III:4) because there is a family history of this disorder. The pedigree is shown
below.

Which one of the following management options is the most appropriate?

A. Amniocentesis for determination of sex, and foetal blood sample for factor VIII level.

B. Chorionic villus sampling (CVS) for determination of sex.

C. CVS, and mutation analysis of the haemophilia A gene if foetus is male.

D. Factor VIII level on mother to determine carrier status.

E. No investigations are necessary.

Question 14

In the presence of meconium-stained liquor, meconium aspiration syndrome is best prevented by:

A. inspection of the vocal cords, followed by intubation and suction if meconium is visualised.

B. intubation and endotracheal suctioning before the first breath.

C. intubation and endotracheal suctioning in the presence of thick meconium-stained liquor.

D. intubation, endotracheal suction and the administration of artificial surfactant.

E. thorough suctioning of the oropharynx before the first breath.

Question 15

A child presents to the Emergency Department in diabetic ketoacidosis.

Following institution of therapy, the most likely cause of death is:

A. acidosis.

B. acute renal failure.

C. cerebral oedema.

D. hypoglycaemia.

E. hypokalaemia.

Copyright © 2003 by The Royal Australasian College of Physicians


9 P201
Question 16

A three-year-old oncology patient is being treated with chemotherapy.

Which one of the following chemotherapy drugs, on extravasation, is most likely to have caused the
appearance as shown above?

A. Cyclophosphamide.

B. Cytosine arabinoside.

C. Ifosfamide.

D. Methotrexate.

E. Vincristine.

Question 17

A four-year-old girl presents with a history of normal development up to nine months of age, followed by a
progressive loss of acquired skills and the onset of repetitive wringing of her hands.

In order to make a clinical diagnosis of Rett syndrome the child should also have:

A. characteristic speech, with clear words but poor cognitive content.

B. characteristic unilateral skin pigmentation.

C. congenital microcephaly.

D. poor head growth starting after six months.

E. typical retinal changes.

Copyright © 2003 by The Royal Australasian College of Physicians


10 P201
Question 18

Which one of the following immunomodulatory therapies is least effective in the management of atopic
dermatitis?

A. Allergen immunotherapy.

B. Azathioprine.

C. Cyclosporin.

D. Interferon gamma.

E. Psoralen and ultraviolet light photochemotherapy.

Question 19

A three-year-old girl presents febrile with an orbital cellulitis, an eye that is discharging pus and tachypnoea.
Her chest X-ray is shown above. An initial intracranial computed tomography (CT) scan is normal. A repeat
CT scan with contrast (shown below) is performed three days later, after a focal convulsion.

The organism most likely to be involved is:

A. Haemophilus influenzae type b.

B. herpes simplex virus.

C. Neisseria meningitidis.

D. Staphylococcus aureus.

E. Streptococcus pneumoniae.

Copyright © 2003 by The Royal Australasian College of Physicians


11 P201
Question 20

A six-month-old girl develops a chylothorax post-cardiac surgery. After two days her chest drain continues to
drain chylous fluid.

The most appropriate feed for the management of chylothorax in this infant is:

A. breast milk.

B. Isomil (soy milk formula).

C. Neocate (single amino acid infant formula).

D. Portagen (casein hydrolysate formula).

E. S26 (whole protein cow’s milk formula).

Question 21

A 10-day-old boy, born at term, is brought to the Emergency Department with a three-day history of lethargy,
poor feeding and vomiting. He is breastfed, but commenced supplemental formula feeds the day prior to the
presentation. His birth weight was 3005 g, head circumference was 34 cm and length was 49 cm.

On examination he is afebrile and mildly dehydrated. His weight is 2650 g. He has episodes of hypertonicity
with opisthotonic posturing and cycling movements of his limbs. Abdominal, cardiovascular and respiratory
examinations are normal.

Investigations show:
haemoglobin 156 g/L [135-205]
9
white cell count 9.5 x 10 /L [5.0-15.0]
9
platelet count 299 x 10 /L [250-450]
sodium 145 mmol/L [135-145]
chloride 105 mmol/L [95-110]
potassium 3.5 mmol/L [3.5-5.5]
bicarbonate 19 mmol/L [22-26]
urea 6.5 mmol/L [1.3-6.6]
creatinine 0.045 mmol/L [0.020-0.050]
lactate 2.2 mmol/L [0.7-2.0]
ammonia 75 µmol/L [<60]
calcium 2.20 mmol/L [2.10-2.75]
glucose 1.9 mmol/L [3.0-5.5]
pH 7.39 [7.35-7.45]
PCO2 35 mmHg
PO2 100 mmHg
cerebrospinal fluid (CSF) glucose 3.4 mmol/L [>3.0]
CSF protein 0.9 g/L [<1.2]
urinalysis ++++ ketones

Cerebral ultrasound demonstrated small ventricles suggestive of cerebral oedema.

Which one of the following is the most likely diagnosis?

A. Isovaleric acidaemia.

B. Maple syrup urine disease.

C. Medium-chain acyl-CoA dehydrogenase deficiency.

D. Ornithine transcarbamylase deficiency.

E. Phenylketonuria.

Copyright © 2003 by The Royal Australasian College of Physicians


12 P201
Question 22

An eight-year-old girl presents with a six-month history of central chest pain. She describes the pain as
squeezing in nature. The pain occurs most days and can occur both at rest and with exercise. She is a
competitive swimmer and has not noted the pain whilst racing. As an infant she was diagnosed on clinical
grounds with a small muscular ventricular septal defect but is now clinically normal.

Echocardiography is most likely to show which one of the following?

A. Aberrant origin of the left anterior descending coronary artery from the right coronary artery.

B. Anomalous left coronary artery.

C. L-transposition of the great arteries.

D. Normal heart.

E. Single coronary artery with a branch crossing between the great arteries.

Question 23

A five-year-old boy presents with a short history of cough. His chest X-rays are shown above.

Which one of the following is the most likely diagnosis?

A. Bronchogenic cyst.

B. Cystadenomatoid malformation.

C. Diaphragmatic hernia.

D. Pneumopericardium.

E. Staphylococcal pneumonia.

Copyright © 2003 by The Royal Australasian College of Physicians


13 P201
Question 24

A four-year-old girl with an unremarkable neonatal history presents with irritability, significant pallor and a
palpable spleen. Full blood count shows:

haemoglobin 40 g/L [110-150]


9
white cell count 11.3 x 10 /L [4.0-12.0]
9
platelet count 383 x 10 /L [150-450]

A photograph of the peripheral blood film is shown below.

The most likely diagnosis is:

A. autoimmune haemolytic anaemia.

B. beta thalassaemia major.

C. Blackfan-Diamond syndrome.

D. megaloblastic anaemia.

E. transient erythroblastopenia of childhood.

Question 25

An 18-month-old boy is referred by his general practitioner to a paediatrician for advice about febrile seizures.
The child has had three episodes of febrile convulsions, the first at 13 months of age. On each occasion, two
seizures have occurred in a 24-hour period. Each seizure was brief (less than five minutes) in the setting of a
high fever (greater than 39°C). The child is developing normally and his neurological examination is
unremarkable. The child’s father has a history of febrile seizures.

The risk of epilepsy in this child is approximately:

A. 0.5%.

B. 2%.

C. 5%.

D. 10%.

E. 15%.

Copyright © 2003 by The Royal Australasian College of Physicians


14 P201
Question 26

A 15-year-old girl with known common variable immunodeficiency, who is receiving regular monthly infusions
of intravenous immunoglobulin, presents with a one-month history of weight loss, loose motions without blood
and intermittent epigastric discomfort which has no clear relationship to meals.

In this clinical setting, the most likely diagnosis is:

A. coeliac disease.

B. cryptosporidiosis.

C. giardiasis.

D. intestinal lymphoma.

E. peptic ulcer disease.

Question 27

A 15-month-old girl has not yet had measles-mumps-rubella (MMR) vaccine, but is up-to-date with her other
vaccinations. The mother and local doctor are concerned about the safety of giving MMR vaccine because of
possible egg allergy. On several occasions after eating foods containing egg the child has developed
generalised urticaria without systemic symptoms. There is a family history of atopy and febrile convulsions in
siblings.

Which one of the following is the most appropriate advice?

A. Delay MMR vaccination until the child is two years of age.

B. Vaccinate with MMR.

C. Vaccinate with MMR under cover of dexamethasone and an antihistamine.

D. Withhold MMR vaccination if the child is skin-prick positive to egg.

E. Withhold MMR vaccination indefinitely as the risk of anaphylaxis is too high.

Question 28

A pregnant woman develops an infectious mononucleosis-like illness at 12 weeks gestation, with fever, sore
throat, malaise and lymphadenopathy. She is proven serologically to have acute cytomegalovirus (CMV)
infection.

What is the most likely outcome for the baby?

A. Hepatitis.

B. Microcephaly.

C. Normal baby.

D. Pneumonitis.

E. Sensorineural deafness.

Copyright © 2003 by The Royal Australasian College of Physicians


15 P201
Question 29

An adolescent presents after an episode of deliberate self-harm.

Which one of the following factors is least predictive of the adolescent going on to complete a suicide?

A. Clear suicidal intent.

B. Gender.

C. History of high-risk impulsive behaviours.

D. Lethality of method.

E. Socio-economic status.

Question 30

A 24-month-old boy, hospitalised for treatment of leukaemia, cries for a long period after his mother leaves at
the end of her visits. The mother at first had difficulty leaving her son but she is now visiting him five or six
times per day. She is becoming increasingly distressed. The nursing staff are in disagreement over how to
respond to this situation and seek advice about the most useful intervention.

Which one of the following is the most appropriate next step?

A. Alternate visits of the boy’s father with those of his mother.

B. Arrange a conjoint meeting with the senior nurse and the mother.

C. Ensure he is looked after by one nurse as much as possible.

D. Recommend the mother room-in full-time.

E. Reduce the frequency of the mother’s visits.

Question 31

A 16-year-old intellectually disabled boy, living in a community residential home, is brought to you for re-
evaluation of longstanding epilepsy and autistic features. His seizures have been well controlled over the last
two years. He had early-onset epilepsy and has been given a diagnosis of autism. You notice unusual
fingernails, which are shown below.

Which one of the following is the most likely diagnosis?

A. Fabry disease.

B. Lesch-Nyhan syndrome.

C. Neurofibromatosis.

D. Tuberous sclerosis.

E. Von Hippel-Lindau disease.

Copyright © 2003 by The Royal Australasian College of Physicians


16 P201
Question 32

A newborn presents with the genitalia shown above. The phallic length is 1.8 cm [3.2-5.0].

The most important criterion determining gender assignment in this infant is:

A. a family history of congenital adrenal hyperplasia.

B. parental wishes.

C. the degree of hypospadias.

D. the degree of micropenis.

E. the presence of scrotal gonads.

Question 33

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 34

An 18-month-old child presents with a confirmed urinary tract infection.

The risk of long-term renal hypertension is best assessed by abnormalities on:

A. DMSA (dimercapto succinic acid) scan.

B. DTPA (diethylenetriamine penta-acetic acid) scan.

C. intravenous pyelogram.

D. micturating cysto-urethrogram (MCU).

E. renal ultrasound.

Copyright © 2003 by The Royal Australasian College of Physicians


17 P201
Question 35

A four-month-old infant presents with biphasic stridor since day one of life and an unusual cry, which has
been described as ‘quiet’. The infant sucks and swallows normally. Growth and development are also
normal.

The most likely cause of the stridor is:

A. infantile larynx (laryngomalacia).

B. lingual cyst.

C. subglottic haemangioma.

D. vascular ring.

E. vocal cord lesion.

Question 36

Which one of the following conditions is not associated with Wilms tumour?

A. Aniridia.

B. Beckwith-Wiedemann syndrome.

C. Cryptorchidism.

D. Hemihypertrophy.

E. Fanconi anaemia.

Question 37

Copyright © 2003 by The Royal Australasian College of Physicians


18 P201
Question 37 (continued)

The X-rays of the knees shown (see previous page) of an eight-month-old Sri Lankan boy are most consistent
with:

A. haemophilia.

B. lead poisoning.

C. non-accidental injury.

D. normal variant.

E. vitamin D deficiency.

Question 38

A two-year-old boy presents in the afternoon to the Emergency Department with a four-hour history of
vomiting, pallor and lethargy. He was well the previous day and there was no history of fever, rash or feeling
hot. The family live in an old house with peeling paint and the social circumstances are poor.

On examination he is pale, unwell and has no rash. He is peripherally cool with a thready pulse. His axillary
o
temperature is 35.8 C, heart rate is 120/minute and blood pressure is 100/70 mmHg. His respiratory rate is
56/minute. Breath sounds are normal. His abdomen is rigid and no bowel sounds can be heard. The X-ray
shown below is taken.

Which one of the following is the most likely diagnosis?

A. Battery ingestion.

B. Iron overdose.

C. Lead poisoning.

D. Mercury poisoning.

E. Pancreatitis.

Copyright © 2003 by The Royal Australasian College of Physicians


19 P201
Question 39

An 18-month-old boy has recurrent blue breath-holding episodes, followed on one occasion by a 15-second
generalised seizure.

The investigation most likely to be useful in directing therapy is:

A. electrocardiogram (ECG).

B. electroencephalogram (EEG).

C. iron studies.

D. plasma calcium.

E. plasma glucose.

Question 40

A child weighing 24 kg with a known, small, perimembranous ventricular septal defect is to undergo routine
isolated dental ‘cleaning and scaling’.

Assuming the child is not allergic to penicillin, which one of the following is recommended in relation to
bacterial endocarditis prophylaxis?

A. Amoxycillin 1.2 g, per oral, administered one hour before the procedure only.

B. Amoxycillin 600 mg, intravenously, administered one hour before the procedure and repeated six
hours after the procedure.

C. Amoxycillin 600 mg, q8h, per oral, for 48 hours prior to the procedure.

D. Antibiotic prophylaxis is not recommended for this procedure.

E. Phenoxymethylpenicillin 1.2 g, per oral, administered one hour before the procedure.

Question 41

Which feature distinguishes hyper-IgE syndrome from severe atopic dermatitis?


+
A. Decreased number of peripheral blood CD8 lymphocytes.

B. Distribution of the eczematous rash.

C. Extremely high serum IgE levels.

D. Negative delayed skin tests to Candida.

E. Staphylococcal abscesses in axillary lymph nodes.

Question 42

Stevens-Johnson syndrome is most likely to occur with which one of the following anticonvulsant drugs?

A. Carbamazepine.

B. Lamotrigine.

C. Phenytoin.

D. Sodium valproate.

E. Vigabatrin.

Copyright © 2003 by The Royal Australasian College of Physicians


20 P201
Question 43

A full term healthy newborn boy is found to have numerous cutaneous petechiae on routine examination at 36
hours of age. Full blood count shows:

haemoglobin 180 g/L [140-200]


nucleated red cells 2/100 white cells [0-10]
9
white cell count 10.6 x 10 /L [6.0-16.0]
9
platelet count 15 x 10 /L [140-450]

Part of his skeletal survey is shown below.

The most likely diagnosis is:

A. amegakaryocytic thrombocytopenia.

B. congenital toxoplasmosis.

C. Fanconi anaemia.

D. Holt-Oram syndrome.

E. thrombocytopenia with absence of the radii.

Copyright © 2003 by The Royal Australasian College of Physicians


21 P201
Question 44

The clinical photograph shown above was taken soon after birth. The infant was delivered vaginally at 34
weeks gestation after premature onset of labour. Birth weight was 2800 g (>90th percentile).

The lesion was covered with a polythene film, and intravenous 10% dextrose was commenced shortly after
delivery. At one hour of age the infant’s core temperature was 35.8°C and the plasma glucose was 0.8
mmol/L.

Which one of the following is the most likely cause for the plasma glucose reading?

A. Abnormal insulin secretion.

B. Cold stress.

C. Defect in fatty acid oxidation.

D. Delayed serum cortisol response.

E. Impaired glycogenesis.

Question 45

A 15-month-old boy is referred for persistent collapse of the right middle lobe, which has been present for
eight weeks following confirmed adenoviral pneumonia. There is no improvement after six weeks of
bronchodilators, oral steroids and physiotherapy. Fine crackles are heard throughout the right lung field.
Investigations reveal a mild iron deficiency anaemia, normal white cell count, normal immunological profile,
normal sweat chloride, negative Mantoux test and a bronchoscopy does not reveal any foreign body or
obvious mucosal lesions.

Which one of the following is the most likely diagnosis?

A. Asthma.

B. Chronic bronchiolitis of infancy.

C. Necrotising pneumonia.

D. Obliterative bronchiolitis.

E. Organising pneumonia.

Copyright © 2003 by The Royal Australasian College of Physicians


22 P201
Question 46

Persistent reduction of serum complement component 3 (C3) is most strongly associated with which one of
the following forms of glomerulonephritis?

A. Diffuse proliferative systemic lupus erythematosus (SLE).

B. Idiopathic membranous.

C. Mesangial IgA disease.

D. Mesangiocapillary.

E. Post-streptococcal.

Question 47

A 13-month-old boy presented with a three-week history of daily fevers and persistent mouth ulcers, and two
days of vomiting and diarrhoea. On examination he was miserable, febrile to 38.9ºC and drooling. The gingiva
were red and friable with multiple shallow grey-coloured ulcers which did not involve the lips, palate or
pharynx. He had multiple small cervical lymph nodes.

Initial investigations revealed:


haemoglobin 89 g/L [95-140]
9
white cell count 11.3 x 10 /L [5.0-17.0]
9
neutrophils 0.2 x 10 /L [1.0-8.0]
9
lymphocytes 9 x 10 /L [2-13]
9
platelet count 614 x 10 /L [150-500]
ESR 7 mm/hr [2-20]
urine culture negative
blood culture negative

The ulcers were swabbed for viral culture, but no virus was isolated.

The fever, vomiting and diarrhoea settled within three days, the mouth ulcers had healed after six days and
the neutrophil count returned to normal after 10 days.

A diagnosis of probable herpes simplex virus infection associated with viral-induced neutropenia was made.

He re-presents two weeks later with a fever and erythema and swelling of the right eye, secondary to
preseptal cellulitis.

Serum immunoglobulin levels are:


IgG <0.33 g/L [3.55-12.10]
IgA <0.07 g/L [0.15-1.24]
IgM 0.92 g/L [0.38-1.66]

Antibodies to tetanus, diphtheria and Haemophilus influenzae type b are not detected despite his
immunisations being up-to-date. T and B cell numbers are normal and T cell proliferation in response to
concanavalin A is normal.

This pattern of immunodeficiency fits best with:

A. chronic granulomatous disease.

B. hyper-IgM syndrome.

C. severe-combined immunodeficiency.

D. transient hypogammaglobulinaemia.

E. X-linked hypogammaglobulinaemia.

Copyright © 2003 by The Royal Australasian College of Physicians


23 P201
Question 48

A 15-year-old girl lives in a rural town where you consult once per month. She presents with a 12-month
history of anxiety-based symptoms, which have prevented her from attending school.

She describes a sense of dread if she is away from home, associated with palpitations, sweating and a heavy
feeling in her chest. She finds it difficult to fall asleep and has numerous nightmares. She worries about
germs and frequently washes her hands. She also describes magical thoughts in that she dreads something
bad will happen if she tapes over her old videos. She believes that these thoughts are silly.

Prior to the onset of her symptoms, she functioned well at school and had many friends. She was not aware
of any pressures at the time.

She lives with her father and a 20-year-old sister, her mother having died from a cerebral haemorrhage eight
years ago. There is no family history of psychiatric illness. She refuses to see a psychiatrist but is willing to
continue to see you.

In addition to further counselling, which one of the following medications is most appropriate?

A. Amitriptyline.

B. Clomipramine.

C. Oxazepam.

D. Paroxetine.

E. Thioridazine.

Question 49

In which one of the following situations is a newborn baby at highest risk of morbidity or mortality from
varicella-zoster virus (VZV) infection?

A. Mother develops chickenpox three days after delivery.

B. Mother develops chickenpox three days before delivery.

C. Mother develops shingles (zoster) five days after delivery.

D. Mother has chickenpox 10 days before delivery, baby born with chickenpox lesions.

E. Two-year-old sibling develops chickenpox when seven-day-old baby is at home, mother seronegative
for VZV.

Question 50

An 18-month-old boy presented with developmental regression following a viral illness. Over the next 18
months he regressed further with periods of relative stability and occasional rapid decline associated with viral
illnesses.

By three years of age, he is unable to walk and has truncal ataxia when sitting. His fine motor skills and
speech are appropriate for a 10 to 12-month-old child. Other features include hypotonia, episodes of unusual
panting respirations and bilateral optic atrophy. Very long chain fatty acids are normal.

Investigations reveal:

blood lactate 2.5 mmol/L [0.7-2.0]


cerebrospinal fluid (CSF) lactate 2.6 mmol/L [<2.5]

Copyright © 2003 by The Royal Australasian College of Physicians


24 P201
Question 50 (continued)

The magnetic resonance imaging (MRI) scan of the brain is shown below.

The most likely diagnosis is:

A. Batten disease.

B. Friedreich disease.

C. Leigh disease.

D. mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes MELAS).

E. metachromatic leukodystrophy.

Question 51

A 12-year-old boy with spastic quadriplegia presents with a 24-hour history of vomiting. A gastrostomy tube
was inserted 12 months ago for supplementary enteral feeding. Currently he has a balloon gastrostomy tube
in situ. Over the past 24 hours the patient has become uncomfortable with oral drinks and solids, but has
tolerated gastrostomy tube feeds. This discomfort appears to be relieved by vomiting. A barium study is
performed (shown below).

Copyright © 2003 by The Royal Australasian College of Physicians


25 P201
Question 51 (continued)

The most likely cause of the vomiting in this boy is:

A. extravasation of feeds into the peritoneal cavity.

B. gastric erosion secondary to irritation of the gastrostomy tube tip.

C. migration of the gastrostomy balloon into the stomal tract.

D. migration of gastrostomy tube into the duodenum.

E. oesophageal stricture.

Question 52

A four-month-old boy presents with significant oozing of blood from the wound following a talipes repair. He is
fully breastfed and previously well with no individual or family history of bleeding.

Investigations show:

haemoglobin 45 g/L [95-140]


prothrombin time-international normalised ratio (PT-INR) 1.2 [1.0-1.4]
activated partial thromboplastin time (APTT) 120 seconds [25-35]

The most likely diagnosis is:

A. factor VIII deficiency.

B. factor X deficiency.

C. factor XI deficiency.

D. vitamin K deficiency.

E. von Willebrand disease.

Question 53

A five-year-old girl presents with constant wetting (day and night). She voids normally but her underpants are
always damp. Physical examination is normal. Urine culture is clear.

Which one of the following is the most likely diagnosis?

A. Depression.

B. Detrusor instability.

C. Diabetes insipidus.

D. Ectopic ureter.

E. Maturational delay.

Copyright © 2003 by The Royal Australasian College of Physicians


26 P201
Question 54

A 13-year-old girl presents with 18 months of migraines, with episodes of transient left hemiparesis. She has
been tearful and missed considerable amounts of school, which she attributes to extensive bullying. Her
mother agrees and believes that the school has managed the situation poorly. Her father is a general
practitioner and mother is a retired nurse and they have previously consulted several colleagues regarding
their daughter’s condition. Extensive investigations have been normal, including a cranial magnetic
resonance imaging (MRI) scan, carotid angiograms and EEG.

After the migraines did not respond to conventional treatment, the neurologist prescribed sertraline in an
appropriate dose for three months. Her difficulties persist and her parents are desperate for her to return to
school.

Which one of the following is likely to be most useful to resolve her problems?

A. Change antidepressant.

B. Change schools.

C. Family therapy.

D. Hypnotherapy.

E. Start sumatriptan.

Question 55

A 34-week gestation infant with severe respiratory distress syndrome was commenced on high frequency
oscillatory ventilation (HFOV) at two hours of age, following surfactant administration.

At 14 hours of age, following a second dose of surfactant, an arterial blood gas revealed the following:
pH 7.45
PaCO2 35 mmHg
PaO2 95 mmHg
base deficit 0
mean arterial blood pressure 45 mmHg
pulse rate 150/minute

The HFOV settings were:


mean airways pressure 16 cm H2O
amplitude 30 cm H2O
frequency 10 Hz
FiO2 0.6

At 16 hours of age there was a deterioration in the infant’s condition. On the same HFOV settings as
previously the arterial blood gas revealed the following:
pH 7.25
PaCO2 50 mmHg
PaO2 40 mmHg
base deficit 1
mean arterial blood pressure 35 mmHg
pulse rate 190/minute

A chest X-ray showed no sign of air leak.

Which one of the following manipulations to the HFOV settings is most likely to produce an improvement in
gas exchange?

A. Decrease the mean airways pressure.

B. Increase the amplitude.

C. Increase the frequency.

D. Increase the inspired oxygen concentration (FiO2).

E. Increase the mean airways pressure.

Copyright © 2003 by The Royal Australasian College of Physicians


27 P201
Question 56

A four-month-old, exclusively breastfed, male infant presents with a three-week history of irritability, poor
feeding and a generalised rash (shown above). The rash is symmetrically distributed and involves the
perioral, acral and perineal areas. His mother suffers from extensive Crohn disease and is taking oral
corticosteroids.

Which one of the following is the most likely diagnosis?

A. Mucocutaneous candidiasis.

B. Psoriasis.

C. Seborrhoeic dermatitis.

D. Vitamin B12 deficiency.

E. Zinc deficiency.

Copyright © 2003 by The Royal Australasian College of Physicians


28 P201
Question 57

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 58

Which one of the following antibiotics is least effective against Pseudomonas aeruginosa infections?

A. Aztreonam.

B. Cefotaxime.

C. Gentamicin.

D. Imipenem.

E. Piperacillin.

Copyright © 2003 by The Royal Australasian College of Physicians


29 P201
Question 59

A five-year-old boy has been rescued from a house fire. He is not cyanosed but has mild inspiratory stridor, a
cough, burns to the face and carbon deposits around the mouth and nose. Cervical spine injury is excluded.
An intravenous cannula has been inserted and he is being given oxygen by face mask.

The most urgent next step in his management is to:

A. administer adequate analgesia.

B. administer nebulised adrenaline.

C. administer nebulised steroids.

D. administer volume replacement rapidly.

E. prepare for endotracheal intubation.

Question 60

A 38-week gestation baby boy was delivered to a 32-year-old woman whose blood group is O Rhesus (Rh)-
negative. She has two other children who had no neonatal problems, and a subsequent termination of
pregnancy. The infant, birth weight 3125 g, was in good condition after delivery but was noted to be slightly
oedematous. Two breastfeeds were given within hours of birth. At nine hours of age he was found to be
tachypnoeic, and on examination was noted to be pale, icteric and generally oedematous. Investigations
revealed:

haemoglobin 95 g/L [125-205]


9
white cell count 3.0 x 10 /L [5.0-19.5]
9
platelet count 41 x 10 /L [150-400]
plasma glucose 4 mmol/L [3-8]
total bilirubin 250 µmol/L
unconjugated 80 µmol/L
conjugated 170 µmol/L
gamma glutamyltransferase (GGT) 129 U/L [0-225]
alanine aminotransferase (ALT) 87 U/L [0-55]
total protein 45 g/L [45-70]
albumin 17 g/L [23-43]

Chest X-ray showed a normal heart size with clear lung fields.

Which one of the following is the most likely diagnosis?

A. Congenital cytomegalovirus (CMV) infection.

B. Foeto-maternal haemorrhage.

C. Galactosaemia.

D. Parvovirus B19 infection.

E. Rhesus iso-immunisation.

Copyright © 2003 by The Royal Australasian College of Physicians


30 P201
Question 61

The father of a five and a half-year-old boy has noticed that for the past four weeks his son has been walking
slowly to school, avoiding stepping on the cracks in the pavement. The boy has always been fussy about
what he eats, wears and plays with. He was clingy and cried for several days at the start of school two
months earlier. He still does not like school and, although other children have been friendly, he has not made
a best friend.

Which one of the following is the most likely diagnosis?

A. Asperger disorder.

B. Depression.

C. Normal child.

D. Obsessive-compulsive disorder.

E. Separation anxiety disorder.

Question 62

The full term infant, shown above at age 36 hours, was born to a primigravida. The pregnancy was
complicated by polyhydramnios and poor foetal movements. The infant required intubation and ventilation
from birth for poor respiratory effort. He remained floppy and had few spontaneous movements.

Which one of the following is most likely to provide diagnostic information?

A. Creatine kinase levels.

B. Electromyography (EMG).

C. Magnetic resonance imaging (MRI) scan of the brain.

D. Muscle biopsy.

E. Triplet repeat studies.

Copyright © 2003 by The Royal Australasian College of Physicians


31 P201
Question 63

The cytokine likely to be responsible for proliferation of the cells indicated in the photograph above is:

A. granulocyte colony-stimulating factor (G-CSF).

B. granulocyte-monocyte colony-stimulating factor (GM-CSF).

C. interleukin 1 (IL-1).

D. interleukin 2 (IL-2).

E. interleukin 5 (IL-5).

Question 64

A 14-year-old healthy girl presents with delayed puberty. Her mother measures 165 cm (50th percentile) and
had her menarche at 13 years of age. Her father measures 177 cm (50th percentile). He was a ‘late
developer’ and did not start shaving until 17 years of age.

On examination the girl’s height is 143 cm (1st percentile), height velocity for the past 12 months is 3 cm/year
(40th percentile) and weight is 52 kg (50th percentile). She is not dysmorphic and has Tanner stage 1 breast
development and stage 2 pubic hair development.

Which one of the following tests is most likely to establish a diagnosis?

A. Anti-endomysial antibody.

B. Bone age.

C. Insulin tolerance test for growth hormone.

D. Karyotype.

E. Plasma oestradiol.

Copyright © 2003 by The Royal Australasian College of Physicians


32 P201
Question 65

A three-year-old boy presents with a swelling under his right jaw, which his parents first noticed a month ago,
and which seems to be slowly enlarging. He has not had fevers, weight loss or malaise and is eating normally.
He has not travelled overseas. The family has a cat, which occasionally scratches him, and two parakeets.

On examination, the boy is afebrile and looks well. He has rubbery nodes, about 3-4 cm in diameter, palpable
in the right submandibular region, which are non-tender and non-fluctuant. The overlying skin has a faint
purple colouration. There is no other lymphadenopathy and the remainder of the examination is normal.

Which one of the following is the most likely diagnosis?

A. Actinomycosis.

B. Atypical mycobacterial infection.

C. Bacterial lymphadenitis.

D. Cat-scratch disease.

E. Tuberculosis.

Question 66

A three-day-old baby, not obviously dysmorphic and weighing 3 kg, presents with mild respiratory distress
and a heart murmur. Subsequent investigation demonstrates truncus arteriosus and low serum calcium.

Which one of the following is the most likely diagnosis?

A. Congenital rubella syndrome.

B. Foetal alcohol syndrome.

C. Foetal hydantoin syndrome.

D. Velocardiofacial syndrome.

E. Williams syndrome.

Question 67

A 10-year-old boy is referred with a 10-day history of headaches, malaise and fever coupled with an
increasingly severe cough and shortness of breath on exercise. He has no significant past medical history.

Examination reveals a flushed, febrile boy with a temperature of 38.4°C orally. There is mild tachypnoea at
rest but no retraction and no use of accessory muscles. Auscultation reveals widespread crackles. His throat
is red and there is a left otitis media, but no other abnormality on examination. His chest X-ray is shown
below.

Copyright © 2003 by The Royal Australasian College of Physicians


33 P201
Question 67 (continued)

The most appropriate oral antibiotic would be:

A. amoxycillin-clavulanic acid.

B. cefaclor.

C. ciprofloxacin.

D. penicillin.

E. roxithromycin.

Question 68

On 32-week antenatal scanning, a cystic right kidney is noted. Four days following delivery, the baby has a
renal ultrasound that shows a left kidney with normal appearances which is on the 95th percentile for length.
The right kidney is less than the 5th percentile in length and contains multiple cysts with no intervening normal
renal parenchyma.

The MAG-3 nuclear imaging clearance study is shown below. Images are taken from the posterior aspect of
the patient. The four images shown at the top are taken before frusemide is administered at one, five, 10 and
15 minutes. The remaining four images are taken after the administration of frusemide at five, 10, 15 and 20
minutes.

L R

The most likely diagnosis is:

A. bilateral polycystic kidney disease.

B. left-sided pelviureteric junction obstruction.

C. left vesicoureteric junction obstruction.

D. right multicystic dysplastic kidney.

E. right pelviureteric junction obstruction.

Copyright © 2003 by The Royal Australasian College of Physicians


34 P201
Question 69

A two-year-old girl was seen by her general practitioner for a febrile illness and was diagnosed as having
exudative tonsillitis and prescribed five days of cefaclor. She had received this antibiotic twice previously
without problems.

She completed her antibiotics and her fever resolved over three days. Two days after stopping the
medication she developed an itchy rash with facial swelling (shown below), painful swelling of her wrists and
ankles and refused to walk.

What is the most likely cause of her rash and arthritis?

A. Adverse drug reaction.

B. Epstein-Barr virus infection.

C. Group A streptococcal infection.

D. Kawasaki disease.

E. Parvovirus B19 infection.

Question 70

A full term infant, birth weight 2800 g, was delivered by normal vaginal delivery to a 19-year-old primigravida
with a history of narcotic addiction. Bottle-feeding with artificial formula was established uneventfully and
meconium was passed at six hours of age.

At three days of age two minor episodes of bile-stained vomiting occurred. The infant’s feeding pattern and
behaviour had been normal. There had been no change in the pattern and nature of the bowel actions.
Examination revealed a well baby whose weight was 2660 g. Abdominal examination revealed no
abnormalities. An abdominal X-ray showed a normal gas pattern.

Which one of the following is the most likely diagnosis?

A. Cow’s milk intolerance.

B. Duodenal stenosis.

C. Gastro-oesophageal reflux.

D. Malrotation.

E. Neonatal narcotic abstinence.

Copyright © 2003 by The Royal Australasian College of Physicians


35 P201
Question 71

Which one of the following vaccines is not recommended for immunocompromised patients?

A. Influenza vaccine.

B. Injectable polio vaccine (Salk).

C. Meningococcal polysaccharide vaccine.

D. Pneumococcal polysaccharide vaccine.

E. Varicella vaccine.

Question 72

Which one of the following would not be consistent with a diagnosis of night terrors?

A. Associated somnambulism.

B. Associated sweating and pupillary dilatation.

C. Inability to recall the event.

D. Multiple episodes per night.

E. Occurrence at age seven years.

Question 73

A five-year-old boy, with a diagnosis of choreoathetoid cerebral palsy, presents with a four-month history of
hostility and aggression towards his siblings. A photograph of his lips is shown below.

The most likely diagnosis is:

A. homocystinuria.

B. Leigh disease.

C. Lesch-Nyhan disease.

D. Menke disease.

E. Wilson disease.

Copyright © 2003 by The Royal Australasian College of Physicians


36 P201
Question 74

A seven-year-old boy meets the diagnostic criteria for attention deficit hyperactivity disorder. You commence
him on a trial of dexamphetamine (2.5 mg at 8 a.m. and 2.5 mg at 1 p.m.).

His mother reports that after one week both she and his school teacher have noted a marked improvement in
attention and task completion, such that his teacher describes him as ‘a different child’. However, his mother
is concerned that his appetite has decreased markedly and he is now taking more than an hour to get to sleep
at night.

Which one of the following is the most appropriate next step in management?

A. Add an appropriate dose of clonidine at night.

B. Cease medication.

C. Change his medication to methylphenidate.

D. Continue the same dose of dexamphetamine.

E. Decrease the dose of dexamphetamine.

Question 75

A seven-year-old boy presents to the Emergency Department two hours after falling over the handlebar of his
bicycle. He complains of mild abdominal pain and is vaguely tender in the epigastrium.

Which one of the following is likely to be the earliest sign of a significant abdominal bleed?

A. Capillary refill time of 5 seconds.

B. Decreased urine output.

C. Hypotension.

D. Reduced pulse volume.

E. Tachycardia.

Question 76

Copyright © 2003 by The Royal Australasian College of Physicians


37 P201
Question 76 (continued)

A six-month-old boy with Down syndrome was diagnosed at birth with a complete atrioventricular canal
defect. He is asymptomatic, growing appropriately and has normal oxygen saturation in room air. On
examination he has a right ventricular heave and a single second heart sound of increased intensity. His
ECG is shown (see previous page).

Which one of the following is the most appropriate course of action?

A. Instigate captopril therapy.

B. Instigate digoxin therapy.

C. Instigate diuretic therapy.

D. Refer for surgical correction.

E. Review in three months.

Question 77

A five-year-old boy presents with a two-month history of progressive ataxia and headaches. A contrast-
enhanced computed tomography (CT) scan taken on hospital admission is shown below.

The most likely diagnosis is:

A. astrocytoma.

B. brain stem glioma.

C. ependymoma.

D. glioblastoma multiforme.

E. medulloblastoma.

Copyright © 2003 by The Royal Australasian College of Physicians


38 P201
Question 78

A 12-year-old girl undergoes gastroscopic examination for persistent epigastric pain. A nodular antral gastritis
is found (shown above). Curved gram-negative bacilli are seen on histology of the antral biopsy.

Which gastrointestinal malignancy has been causally linked with this condition?

A. Adenocarcinoma of the gastric cardia.

B. Adenocarcinoma of the oesophagus.

C. Gastric lymphoma.

D. Malignant gastrinoma.

E. Squamous carcinoma of the oesophagus.

Question 79

A 16-year-old boy presents with a history of three episodes of low back pain over six months, each lasting for
10 days. The pain is worse later in the day, eased by rest, and is not associated with morning stiffness in the
back.

Investigations show:

full blood examination within normal limits


ESR 19 mm/hr [2-20]
HLA-B27 positive
X-ray of lumbosacral spine and sacro-iliac joints normal

The most likely diagnosis is:

A. ankylosing spondylitis.

B. discitis.

C. non-specific low back pain.

D. Scheuermann’s osteochondritis.

E. spondylolysis.

Copyright © 2003 by The Royal Australasian College of Physicians


39 P201
Question 80

A 15-year-old boy has been on an anticonvulsant for three years. The dosage has been stable. He recently
noticed difficulties in seeing at night. A formal ophthalmological assessment demonstrated visual field
constriction.

Which one of the following anticonvulsants is most likely to be the cause of his symptoms?

A. Carbamazepine.

B. Gabapentin.

C. Lamotrigine.

D. Sodium valproate.

E. Vigabatrin.

Question 81

A three-year-old boy presents with a three-day history of complaining of a ‘sore bottom’, which is intensely
itchy. He complains particularly of pain on defaecation and has a mucopurulent anal discharge. His anal
appearance is shown below.

Which one of the following is the most likely diagnosis?

A. Candidiasis.

B. Child sexual abuse.

C. Group A streptococcal infection.

D. Pruritus ani.

E. Threadworm infestation.

Copyright © 2003 by The Royal Australasian College of Physicians


40 P201
Question 82

You are asked to assess a 13-year-old girl who can swim 2 km without difficulty but cannot run more than 50
m without stopping for shortness of breath. There is a history of coughing illnesses in earlier childhood,
treated with salbutamol. She uses salbutamol before running.

Her cardio-respiratory examination is normal. Her weight is on the 75th percentile and her height is on the
50th percentile. She has Tanner stage 2 breast development. Her lung function tests reveal a normally
shaped flow volume loop, the forced expiratory volume in 1 second (FEV1) is 87% predicted and there is no
response to bronchodilators. A hypertonic saline challenge does not result in any change in her lung function.

The area your consultation should concentrate on to best explain her difficulties is:

A. drug delivery.

B. exercise-induced asthma.

C. motivation difficulties.

D. muscle fatigue/fitness.

E. radiographic assessment.

Question 83

A two and a half-year-old girl is referred for assessment of her gait. Her parents have noted that she trips and
falls many times each day and experiences considerable difficulty with running. History includes a normal
pregnancy, delivery and neurodevelopmental milestones. There is no family history of musculoskeletal
disorders.

Examination shows a markedly intoeing gait with normal feet. There is tibial bowing and some anteversion of
the femoral neck bilaterally. There is no abnormality of muscle power, tone or joint movement. Neurological
examination is normal and her height and weight are on the 75th percentile.

The most appropriate recommendation is:

A. night splinting of the lower limbs.

B. physiotherapy assessment.

C. podiatry assessment.

D. radiological assessment of the lower limbs.

E. reassurance.

Question 84

A four-week-old infant presents with tachypnoea and failure to thrive. On examination the infant has a
respiratory rate of 60/minute with subcostal recession. The pulses are full and the liver is palpable 4 cm
below the right costal margin. The praecordium is active. On auscultation there is an audible ejection click
and a non-specific systolic ejection murmur is noted at both upper sternal edges. The infant’s oxygen
saturation in room air is 93%.

Which one of the following is the most likely diagnosis?

A. Persistent ductus arteriosus.

B. Pulmonary valve stenosis.

C. Truncus arteriosus.

D. Ventricular septal defect.

E. Ventricular septal defect and severe pulmonary valve stenosis.

Copyright © 2003 by The Royal Australasian College of Physicians


41 P201
Question 85

A 16-year-old girl has had six episodes of nephrotic syndrome over the last five years. The initial episodes
responded promptly and completely to oral prednisolone, but recent episodes have responded more slowly.
At the completion of her last course of prednisolone two weeks ago, urine protein was 500 mg/day.

Her trichrome-stained renal biopsy is shown below (A) with a normal renal biopsy also shown (B).

A B

Which one of the following is the most likely diagnosis?

A. Focal sclerosing glomerulonephritis.

B. IgA nephropathy.

C. Membranous glomerulonephritis.

D. Minimal change glomerulonephritis.

E. Post-streptococcal glomerulonephritis.

Question 86

A 14-year-old Lebanese boy with type 1 (insulin-dependent) diabetes mellitus of two years duration has a
deterioration in his metabolic control. His current insulin dose is 0.8 U/kg/day. He measures his blood
glucose at home using a glucometer. His blood glucose record book over the past week (typical of the past
three months’ readings) is shown below (all values are in mmol/L).

Pre-breakfast Pre-lunch Pre-dinner Pre-bedtime


Monday 4.5 6.3 5.7 8.0
Tuesday 5.6 10.3 6.2 7.0
Wednesday 6.3 5.6 10.3 9.6
Thursday 7.4 8.4 6.9 7.6
Friday 5.8 5.9 8.5 6.8
Saturday 7.1 12.6 5.9 11.5
Sunday 10.5 8.1 6.8 9.4

On examination, his height is 169 cm (75th percentile) and weight is 40 kg (10th percentile, static over the
past 12 months). He has mild lipohypertrophy at his abdominal injection sites. He is in mid-puberty.

His HbA1c readings over the past 12 months have been 8-9% but the most recent reading is 12.6% [non-
diabetic: 4.8-6.0]. The HbA1c repeated using a different assay was 12.4%.

Copyright © 2003 by The Royal Australasian College of Physicians


42 P201
Question 86 (continued)

The most appropriate management response is to:

A. perform haemoglobin electrophoresis.

B. recommend opening new vials of insulin.

C. rotate injection sites.

D. trial metformin in addition to insulin.

E. verify stored glucometer readings.

Question 87

Pre-symptomatic genetic testing is now available for many inherited disorders.

Which one of the following would be the most appropriate reason for testing a nine-year-old child?

A. Medical intervention can alter the disorder’s natural history.

B. Parental anxieties can be relieved.

C. Parents want to take out life insurance on the child.

D. The child wants to be tested.

E. The child’s carrier status can be determined.

Question 88

A 12-year-old male bone marrow recipient develops acute upper abdominal pain on day 10 after
transplantation from a related donor. Over the following three days he develops increasing abdominal
distension and jaundice. He has developed tender hepatomegaly and gained 4 kg in weight. His bowel
sounds are sparse. His liver function tests reveal:

alanine aminotransferase (ALT) 1850 U/L [5-45]


gamma glutamyltransferase (GGT) 683 U/L [5-24]
conjugated bilirubin 125.0 µmol/L [0-3.4]
unconjugated bilirubin 12 µmol/L [0-17]
albumin 30 g/L [40-53]
total protein 55 g/L [66-82]

His plain abdominal film shows marked ascites.

The most likely diagnosis is:

A. acute cholecystitis.

B. graft-versus-host disease of the liver.

C. hepatic veno-occlusive disease.

D. primary peritonitis.

E. transfusion-related acute hepatitis.

Copyright © 2003 by The Royal Australasian College of Physicians


43 P201
Question 89

A seven-year-old boy has three generalised tonic-clonic seizures over a three-week period. His EEG is
shown below.

Which one of the following anticonvulsants is relatively contraindicated?

A. Carbamazepine.

B. Clonazepam.

C. Gabapentin.

D. Lamotrigine.

E. Sodium valproate.

Question 90

A one-month-old boy presents severely shocked. His ECG is shown below.

The most appropriate treatment is:

A. ice water immersion.

B. intravenous adenosine.

C. intravenous verapamil.

D. one-sided carotid body massage.

E. synchronised direct current (DC) shock.

Copyright © 2003 by The Royal Australasian College of Physicians


44 P201
Question 91

A term neonate presents with ‘jitteriness’ at three hours of age. The following recordings are made:

weight 3450 g (50th percentile)


length 50 cm (50th percentile)
head circumference 36 cm (50th percentile)
penile length 1.8 cm (<10th percentile)
plasma glucose 0.7 mmol/L

The remainder of the examination is normal.

The most likely cause is:

A. congenital adrenal hyperplasia.

B. 5 alpha-reductase deficiency.

C. hyperinsulinism.

D. hypopituitarism.

E. 45XO/46XY mosaicism.

Question 92

A mother brings her one-year-old son to see you. For the past three months he has been waking every night,
usually two hours after settling. He falls asleep at the start of the night with a bottle of milk. When he wakes
during the night, he cries, stands up in the cot and drools. When his mother is unable to calm him with patting
she gives him a bottle of 30 mL of formula. He settles back to sleep after drinking the formula. The mother
says he is irritable during the day and this is ‘getting her down’. Physical examination is normal.

Which one of the following is the most likely diagnosis?

A. Hunger.

B. Night terrors.

C. Separation anxiety disorder.

D. Sleep association disorder.

E. Teething.

Question 93

An otherwise well infant is found to have a continuous murmur at the time of delivery. The infant’s oxygen
saturation in room air is 99%.

The most likely diagnosis is:

A. coronary artery fistula.

B. patent foramen ovale.

C. persistent ductus arteriosus.

D. pulmonary atresia.

E. tetralogy of Fallot and absent pulmonary valve syndrome.

Copyright © 2003 by The Royal Australasian College of Physicians


45 P201
Question 94

A 16-year-old girl with pneumonia was given an intravenous injection of penicillin. She developed
hypotension and exhibited difficulty with ventilation.

Immediately following resuscitation, which one of the following tests would be most supportive of a diagnosis
of anaphylaxis?

A. C1-esterase inhibitor level.

B. Penicillin radioallergosorbent test (RAST).

C. Serum elastase.

D. Serum histamine.

E. Serum tryptase.

Question 95

A breastfed five-month-old boy presents with poor feeding and diminished activity over a four-day period. His
parents are first cousins.

On examination the child is listless with little spontaneous movement. He has absent tendon reflexes and
ptosis. His pupils are poorly reactive and dilated.

Which one of the following is the most likely diagnosis?

A. Botulism.

B. Congenital myopathy.

C. Guillain-Barré syndrome.

D. Pompe disease.

E. Spinal muscular atrophy.

Question 96

A male infant was born at 35 weeks gestation following premature rupture of membranes. Hypothermia and
unconjugated hyperbilirubinaemia were problems during the newborn period.

He presents at six months with myoclonic seizures and is found to be globally developmentally delayed. He is
not rolling, does not reach for objects (although he is reported to have done so previously) and does not
vocalise normally. There is generalised mild hypotonia but no focal neurological signs. He has hair which
breaks easily, leaving generally short hair with a stubbly feel to his scalp. His photograph is shown below.
His mother is said to have had similar hair as a child. A maternal uncle had seizures and developmental
delay and died at age three years.

Copyright © 2003 by The Royal Australasian College of Physicians


46 P201
Question 96 (continued)

Which one of the following investigations is most likely to yield a diagnosis?

A. Cranial computed tomography (CT) scan with contrast.

B. Nerve conduction studies.

C. Serum copper levels.

D. Serum zinc levels.

E. Urine metabolic screen.

Question 97

A two and a half-year-old girl presents to the Emergency Department with a short history of an expiratory
wheeze. Her history includes a choking episode at a party three days ago, asthma and probable gastro-
oesophageal reflux as a baby.

On examination she is pink, afebrile, has obvious eczema and is in mild respiratory distress. The examination
also reveals widespread expiratory wheezes which persist after a trial of nebulised salbutamol. Her chest X-
ray is normal.

The most important next step is:

A. bronchoscopy.

B. computed tomography (CT) scan of chest.

C. inspiratory and expiratory chest X-rays.

D. screening of diaphragm.

E. trial of oral corticosteroids.

Question 98

A 15-year-old girl presents to her local doctor with acute tonsillitis. On examination she appears slightly
jaundiced.

Her liver function tests reveal:

total bilirubin 42 µmol/L [0-17]


conjugated bilirubin 0 µmol/L [0-3.4]
alanine aminotransferase (ALT) 15 U/L [5-45]
gamma glutamyltransferase (GGT) 19 U/L [5-24]
alkaline phosphatase (ALP) 125 U/L [70-230]

The most likely diagnosis is:

A. Crigler-Najjar syndrome.

B. Gilbert syndrome.

C. glucose-6-phosphate dehydrogenase deficiency.

D. infectious mononucleosis.

E. Wilson disease.

Copyright © 2003 by The Royal Australasian College of Physicians


47 P201
Question 99

An 18-month-old boy presents with pallor, lethargy and tachycardia. His full blood count shows:

haemoglobin 50 g/L [100-150]


mean corpuscular volume (MCV) 62 fL [80-100]
9
white cell count 4.8 x 10 /L [3.5-12.0]
9
platelet count 520 x 10 /L [150-450]
albumin 22 g/L [25-40]

A photomicrograph of the peripheral blood film is shown below.

The most likely diagnosis is:

A. acute lymphoblastic leukaemia.

B. beta thalassaemia major.

C. glucose-6-phosphate dehydrogenase deficiency.

D. hereditary spherocytosis.

E. iron deficiency anaemia.

Question 100

A previously well two and a half-year-old girl, whose parents are divorced, returns from weekend access with
her father. On return home the child is a little more demanding than usual but is otherwise behaviourally
normal. At bed-time the mother notices that her daughter has a significantly reddened vulva.

Which one of the following is the most likely diagnosis?

A. Candidiasis.

B. Child sexual abuse.

C. Lichen sclerosis.

D. Non-specific vulvovaginitis.

E. Straddle injury.

Copyright © 2003 by The Royal Australasian College of Physicians


48 P201
2001 FRACP Written Examination

Paediatrics & Child Health

Paper 2 – Clinical Applications

Answers

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

Copyright © 2003 by The Royal Australasian College of Physicians

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