Model Paper One

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Model paper one

Question 1.

A rhythm strip from an electrocardiogram (ECG) is shown above. Which


of the following is the most likely cause for the abnormality
displayed?
A. Hyperkalaemia.
B. Hyperthermia.
C. Hyperthyroidism.
D. Hypocalcaemia.
E. Uraemia.

2. Steroids used in the treatment of childhood acute lymphoblastic


leukaemia are least frequently associated with which one of the
following side effects?
A. Avascular necrosis of bone.
B. Behavioural disturbances.
C. Bone demineralisation.
D. Renal tubular defects.
E. Weight gain.

3. The most important known factor which influences the rate of


development of microvascular complications in patients with diabetes
is:
A. duration of diabetes.
B. hyperglycaemia.
C. hyperlipidaemia.
D. hypertension.
E. smoking.

4. Which of the following anticonvulsants is most likely to increase


the half-life of topiramate?
A. Carbamazepine.
B. Phenobarbitone.
C. Phenytoin.
D. Primidone.
E. Sodium valproate.

5. Which of the following antibiotics would be expected to be least


active in treating a patient with infection caused by Pseudomonas
aeruginosa?
A. Cefotaxime.
B. Gentamicin.
C. Imipenem.
D. Piperacillin.
E. Ticarcillin.
6. A 24-day-old boy presents with a one-week history of projectile
vomiting and weight loss. Which of the following findings would be
most likely on arterial blood gas measurement?
A. Metabolic acidosis.
B. Metabolic alkalosis.
C. Mixed metabolic acidosis and respiratory alkalosis.
D. Mixed metabolic alkalosis and respiratory acidosis.
E. Normal blood gas.

7. In a child with a massive abdominal Burkitt lymphoma, the most


useful treatment to prevent tumour lysis syndrome is:
A. allopurinol.
B. frusemide.
C. mannitol.
D. prednisolone.
E. urate oxidase.

8. Idiopathic ascending aortic dilatation would be most likely to


accompany which of the following syndromes/associations?
A. Down syndrome.
B. Noonan syndrome.
C. Turner syndrome.
D. VACTERL association.
E. Velocardiofacial syndrome.

9. A breastfed two-day-old girl presents with profuse diarrhoea and


develops severe dehydration. The diarrhoea ceased during
resuscitation with intravenous (IV) fluids when feeds were paused.
However, when breastfeeds were gradually introduced in the recovery
period, the diarrhoea returned and IV fluids were recommenced. A
trial of oral rehydration solution also resulted in severe
diarrhoea. Analysis of the faecal fluid revealed the following
electrolyte profile:
sodium 20 mmol/L
potassium 15 mmol/L
chloride 25 mmol/L
Which one of the following is the most likely diagnosis?
A. Congenital chloride diarrhoea.
B. Glucose-galactose malabsorption.
C. Primary lactose intolerance.
D. Secondary lactose intolerance.
E. Sucrase-isomaltase deficiency.

10. The daily energy requirement of a parenterally fed three-week-


old neonate is estimated to be 100 kcal (420 kJ) per kilogram body
weight. Which of the following intravenous glucose and lipid
solutions will provide adequate daily energy for an infant of 4 kg
body weight?

A. 400 mL of glucose 10% plus 20 mL of intravenous lipid 20%.


B. 400 mL of glucose 15% plus 30 mL of intravenous lipid 20%.
C. 400 mL of glucose 15% plus 40 mL of intravenous lipid 20%.
D. 400 mL of glucose 15% plus 50 mL of intravenous lipid 20%.
E. 400 mL of glucose 20% plus 60 mL of intravenous lipid 20%.
11. Which of the following antipsychotic medications is most likely
to cause the greatest weight increase when prescribed to
adolescents?
A. Haloperidol.
B. Olanzapine.
C. Pimozide.
D. Quetiapine.
E. Risperidone.

12. Low serum vitamin B12 levels are least likely to be seen in which
one of the following?
A. Crohn disease.
B. Pernicious anaemia.
C. Severe folate deficiency.
D. Short bowel syndrome.
E. Transcobalamin II deficiency.

13. The word ‘complex’ in complex partial seizures refers to:


A. a seizure lasting >10 minutes.
B. automatisms occurring during the seizure.
C. focal motor seizures.
D. impaired consciousness during the seizure.
E. multiple seizures in a 24-hour period.

14. The most effective way of preventing mother-to-child


transmission of human immunodeficiency virus (HIV) is:
A. antiretroviral therapy to the mother during delivery.
B. antiretroviral therapy to the mother during pregnancy.
C. antiretroviral therapy to the newborn baby.
D. bottle-feeding.
E. Caesarean section.

15. Which of the following most accurately describes the known


effects of supplementation of artificial milk formulae with long
chain polyunsaturated fatty acids (LCPUFA) in term infants?
A. A beneficial effect on bone maturation.
B. A beneficial effect on growth.
C. A beneficial effect on neurodevelopmental outcome.
D. A beneficial effect on visual maturation.
E. No additional benefit demonstrated.

16. If two or more first-degree relatives have atopic disorders, the


risk of a subsequent infant developing an atopic disorder is closest
to:
A. <10%.
B. 20%.
C. 33%.
D. 75%.
E. 90%.

17. Activated protein C resistance is most likely to be associated


with which one of the following states?
A. Antithrombin III deficiency.
B. Factor V Leiden mutation.
C. Factor VIII deficiency.
D. Lupus anticoagulant.
E. Prothrombin gene mutation.

18. A six-year-old patient with coeliac disease undergoes dual sugar


intestinal permeability testing. Two sugars, rhamnose and lactulose,
are administered orally and the subsequent urinary excretion is
measured over five hours. Rhamnose is actively absorbed by the small
intestinal villus, whereas lactulose is absorbed via passive
paracellular transport. Which of the following findings in urine
indicate poor adherence to a gluten-free diet?
Lactulose
Rhamnose

A. Decreased Decreased

B. Decreased Normal

C. Increased Decreased

D. Increased Normal

E. Normal Increased

19. An ambulance attends a 13-year-old girl who collapses at school


and is unconscious for a brief period. Witnesses to the event said
that she had some brief jerking of her limbs when on the ground. By
the time she is brought to the emergency room she is back to normal.
On further questioning, there have been three previous events.
An association with which of the following is most likely to raise
concerns that the diagnosis is not simple syncope?
A. Exercise.
B. Mild trauma.
C. Prolonged standing.
D. Venipuncture.
E. Vomiting.

20. Among children and adolescents with significant behavioural


disturbances, a poor prognosis is most likely with which of the
following?
A. Alcoholic or sociopathic father.
B. Greater severity of problem behaviour.
C. Lower age of presentation.
D. Maternal psychosocial problems.
E. Parental divorce.

21. A 12-year-old girl completed treatment for a Ewing sarcoma four


years ago. The chemotherapy agent most likely to increase her risk
of second malignancy is:
A. actinomycin-D.
B. cyclophosphamide.
C. doxorubicin.
D. methotrexate.
E. vincristine.
22. Which one of the following interventions has most consistently
been shown to decrease the duration of hospital stay with acute
asthma?
A. Inhaled ipratropium bromide.
B. Inhaled salbutamol.
C. Intravenous salbutamol.
D. Oral prednisolone.
E. Oral theophylline.

23. 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.

24. 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.

25. 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.
Which one of the following is the most likely diagnosis?
A. Antiphospholipid antibody syndrome.
B. Factor VII deficiency.
C. Haemophilia.
D. Recent aspirin ingestion.
E. Von Willebrand disease.
26. Second generation H1 receptor antagonists effectively relieve
most of the symptoms of allergic rhinoconjunctivitis.
Which one of the following symptoms is least effectively relieved?
A. Nasal congestion.
B. Nasal itch.
C. Ocular tearing.
D. Rhinorrhoea.
E. Sneezing.

27. 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

28. 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.

29. 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.

30. A three-year-old boy presents with a six-month history of


polyarticular arthritis. He had been well until age 12 months when
he required hospitalisation for left lower lobe pneumonia. Recurrent
otitis media, intermittent fevers and persistent diarrhoea developed
in the second year of life. There was no history of oral thrush or
other fungal infections and no history of opportunistic infections.
Developmental milestones and physical growth were normal and
immunisations were appropriate for age.
Physical examination shows a febrile boy who refuses to weight bear.
He has purulent nasal discharge. The lung fields are clear. Both
knees are swollen, tender and warm with effusions and decreased
range of movement. Both ankles are tender with synovial swelling and
there is a reduced range of movement. Examination of the heart is
normal. Skin examination is normal.
Which one of the following is the most likely diagnosis?
A. Chronic meningococcaemia.
B. Juvenile chronic arthritis.
C. Late onset hypogammaglobulinaemia (common variable
immunodeficiency).
D. Rheumatic fever.
E. Yersinia arthritis.

31. 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 the axillary lymph nodes.

32. A four-year-old child has a pneumonia with an effusion that is


unresponsive to antibiotic therapy given intravenously in
appropriate doses (ceftriaxone, tobramycin and flucloxacillin) and
formal surgical drainage of the effusion/empyema. Five days after
the surgery the child still has temperatures greater than 39°C and
looks unwell. Bacteroides fragilis is grown from the effusion.
A change to which one of the following antibiotics is likely to
improve the child?
A. Ceftazidime.
B. Clindamycin.
C. Erythromycin.
D. Penicillin G.
E. Vancomycin.

33. You are asked for a second opinion regarding a 10-year-old boy
who has a long history of hyperactive, impulsive, attention-seeking
and destructive behaviour, both at home and at school. A
paediatrician and a child psychiatrist have previously diagnosed him
as having attention deficit hyperactivity disorder (ADHD). He
responded poorly to dexamphetamine but concentration and attention
have improved on methylphenidate. He is currently on 30 mg/day. His
weight is 35 kg.
Further assessment demonstrated average intelligence but with a
significant language-based learning disorder. He is at the 3rd
percentile for receptive language and the 14th percentile for
expressive language. He is making progress at school with the
provision of remedial help and an integration aide.
He also receives on-going counselling and cognitive behavioural
management from his psychiatrist.
His parents are divorced and are unable to manage him consistently.
His father is strict, distant and loses his temper with his son. His
mother is caring, finds it hard to set limits and is overwhelmed by
his demands. The boy dominates and intrudes on all aspects of family
life. His father denigrates his ex-wife in front of the boy.

Which one of the following is likely to be the most helpful?


A. Alter medication regime.
B. Family therapy.
C. Foster care.
D. Individual parental guidance.
E. Transfer to a special school.
34. A 14-month-old infant has been dependent on parenteral nutrition
since birth due to surgical resection secondary to volvulus of the
small bowel. He currently receives 30% of his caloric requirement by
the parenteral route and the remainder as Pregestimil® via a
nasogastric tube. On examination he is icteric. He has 3 cm of
splenomegaly, the liver is not palpable and there is no ascites.
His blood tests reveal:
bilirubin (total) 120 μmol/L [<10]
bilirubin conjugated 96 μmol/L [<10]
alanine aminotransferase (ALT) 203 U/L [10-50]
gamma glutamyltransferase (GGT) 593 U/L [5-24]
albumin 23 g/L [34-52]
Abdominal ultrasound shows a small liver with normal intra and extra
hepatic ducts and an enlarged spleen. A Doppler study of his portal
vessels shows blood flowing from the liver to the spleen. The most
effective intervention to arrest the progression of his liver
disease would be to:
A. administer chenodeoxycholic acid.
B. administer phenobarbitone.
C. administer rifampicin.
D. cease parenteral nutrition.
E. perform a portacaval shunt.

35. A four-year-old boy presents with the sole symptom of a


generally dry cough of four months duration, which has been
affecting his sleep. He is well grown and there are no localising or
generalised signs. He has not responded to nebulised salbutamol
prescribed by his general practitioner. His blood count and chest X-
ray are normal.
Which one of the following is the most appropriate management?
A. Bedroom humidification.
B. Erythromycin.
C. Inhaled corticosteroids.
D. No therapy.
E. Oral steroids.

36. 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:
haemoglobin 117 g/L [110-150]
mean corpuscular volume 79 fL [75-90]
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.

37. Which one of the following factors is most likely to be


associated with the occurrence of late-onset haemorrhagic disease
(vitamin K deficiency bleeding)?
A. Breastfeeding.
B. Formula feeding.
C. Maternal anticonvulsants.
D. Post-natal antibiotic administration.
E. Prematurity.

38. 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.

39. A 15-year-old girl presents with a three-year history of school


avoidance, attending approximately one week per month. She held a
scholarship in secondary school but this was withdrawn due to poor
attendance.
At home, she spends her day watching television, drinking alcohol or
smoking marijuana. She has a few friends but is reluctant to leave
the house. She is preoccupied with her body and feels herself to be
a freak because she perceives asymmetry in her facial appearance.
She panics in social situations, believing that other people see
this appearance. She is volatile and flies into a rage if she is
thwarted. She hates herself, has grazed her wrists and there are
scars from cigarette burns on her arms. She denies suicidal
ideation.
At night, she finds it difficult to fall asleep, worries about being
attacked and often checks the door and window locks. She has
frequent nightmares and on waking, she thinks she hears strangers in
her room.
Her parents divorced eight years ago and do not get on well. She
lives with her mother and brother, the latter having similar but
less severe difficulties. He is now at university. Her mother is
anxious and ineffectual and it is clear that the children control
the family.
Which one of the following diagnoses best explains this picture?
A. Affective disorder (depression).
B. Borderline personality disorder.
C. Obsessive-compulsive disorder.
D. Panic disorder.
E. Schizophrenia.

40. 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.

41. A 13-year-old boy is brought to the emergency room following an


intentional ingestion of battery acid 15 minutes previously. He
complains of abdominal pain. On examination he is pale with a pulse
rate of 100/minute, respiratory rate of 30/minute and a systolic
blood pressure of 120 mmHg. He has upper abdominal tenderness with
no obvious rebound tenderness. Bowel sounds are present and normal
in character.
Which one of the following is the most appropriate immediate
management?
A. Administer antacid.
B. Administer corticosteroids.
C. Administer ipecac.
D. Insert a nasogastric tube and suction gastric contents.

E. Perform a gastroscopy.

42. 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

43. An eight-month-old breast-fed baby girl is brought to the


emergency department with a 10-day history of vomiting, irritability
and weight loss. The baby developed normally until six months of age
but since then her development has regressed and she is no longer
able to sit unsupported.
She is afebrile, pale and listless. Her weight is 6.5 kg (3-10th
percentile), length 68 cm (50th percentile) and head circumference
is 43 cm (10-50th percentile). She is generally hypotonic and has
brisk reflexes with positive Babinski responses. Abdominal
examination is normal. Investigations show:
haemoglobin 98 g/L [95-140]
mean corpuscular volume 106.5 fL [70.0-85.0]
mean corpuscular haemoglobin 34.9 pg [24.0-36.0]
white cell count 5.8 x 109/L [5.0-17.0]
neutrophils 1.1 x 109/L [1.0-8.0]
occasional hypersegmented neutrophils seen on blood film
platelet count 445 x 109/L [150-600]
sodium 145 mmol/L [135-145]
chloride 99 mmol/L [95-110]
potassium 4.5 mmol/L [3.5-5.5]
bicarbonate 26 mmol/L [22-26]
urea 6.9 mmol/L [1.3-6.6]
creatinine 0.045 mmol/L [0.020-0.050]
glucose 4.3 mmol/L [2.5-5.5]
Urine metabolic screen gross increase in methylmalonic acid and
homocystine
Which one of the following is the most likely diagnosis?
A. Cobalamin C deficiency.
B. Glutaric aciduria type II.
C. Homocystinuria.
D. Methylmalonic acidaemia.
E. Vitamin B12 deficiency.

44. 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.

45. 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%.

46. 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.

47. A 10-year-old girl presents with a recurrent persistent


distressing cough of four months duration. The cough does not occur
during sleep, but starts on awakening and fluctuates in intensity
and frequency throughout the day. Her mother describes the cough as
very loud. The cough has been present daily and it started with an
upper respiratory tract infection. There was a similar episode 12
months ago at the same time of the year. The cough was not
associated with wheeze, dyspnoea or any sputum production. Her chest
X-ray is normal.
Which one of the following is the most likely diagnosis?
A. Cough variant asthma.
B. Episodic asthma.
C. Pertussis.
D. Post-viral chronic bronchitis.
E. Psychogenic cough.

48. 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:
sodium 135 mmol/L [134-142]
potassium 2.7 mmol/L [3.5-4.5]
chloride 98 mmol/L [96-110]
urea 1.0 mmol/L [2.1-6.5]
creatinine 0.02 mmol/L [0.03-0.08]
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]
albumin 30 g/L [35-50]
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.

49. Which one of the following cardiac lesions would be an


unexpected finding in a baby with a 22q11 deletion?
A. Infradiaphragmatic totally anomalous pulmonary venous return.
B. Interrupted aortic arch.
C. Pulmonary atresia, ventricular septal defect and major aorto-
pulmonary collaterals.
D. Tetralogy of Fallot.
E. Truncus arteriosus.

50. Which one of the following measures is most effective in


preventing neonatal early onset group B streptococcal infection?
A. Antibiotics (single intramuscular dose) given after delivery to
babies of colonised mothers.
B. Antibiotics given during pregnancy to colonised mothers at 28
weeks gestation.
C. Antibiotics given during pregnancy to colonised mothers with
group B streptococcal antigenuria.
D. Maternal intrapartum antibiotics to colonised mothers.
E. Vaginal antiseptic douches before and during delivery to
intrapartum colonised mothers.

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