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Flashcards in 2008 Paper A Deck (70):

Cerebral palsy is defined as a disorder of posture and movement due to an insult to the developing brain. What is the most common period for the responsible brain insult to occur?
A. Antenatal.
B. Intrapartum.
C. Neonatal.
D. 1 to 12 months.
E. After 12 months.

Answer A. Antenatal


A three-year-old boy is being resuscitated in the community following an immersion injury. What is the currently recommended ratio of external cardiac compressions to exhaled air resuscitations for a two rescuer resuscitation in this patient?
A. 3:1
B. 5:1
C. 15:2
D. 30:2
E. 30:1

Answer C. 15:2


In an otherwise well toddler with significant nutritional iron deficiency anaemia, what is the expected time interval between commencing iron therapy and a reticulocytosis becoming evident on a blood film?
A. 3 days.
B. 7 days.
C. 14 days.
D. 30 days.
E. 60 days.

Answer A. 3 days


A six-month-old child with cardiac failure secondary to severe dilated cardiomyopathy is initially treated with Frusemide and Spironolactone. The addition of which of the following drugs to the child’s medical therapy is most likely to result in hyperkalaemia if no changes are made to the existing therapy?
A. Amiodarone.
B. Captopril.
C. Digoxin.
D. Metoprolol.
E. Warfarin.

B  Captopril


Which of the following vaccines is least likely to induce long term immunologic memory?
A. Bacille Calmette-Guérin (BCG).
B. Diphtheria-tetanus-pertussis.
C. Haemophilus influenzae type B conjugate.
D. Hepatitis B.
E. Pneumococcal polysaccharide

E  Pneumococcal polysaccharide


The results of a study undertaken to determine the utility of urinary leukocyte dipstick testing in the diagnosis of urinary tract infections (UTI) in febrile children is shown below.

In this study, the negative predictive value of the leukocyte dipstick test is:
A. 35%
B. 70%
C. 75%
D. 90%
E. 95%

C 75%


An 11-year-old boy is seen in clinic for review of congenital cardiac disease, for which he underwent surgery as a neonate. You notice asymmetry of his face, as demonstrated in the photograph below

These findings are consistent with:
A. Duane syndrome.
B. Horner syndrome.
C. Mobius syndrome.
D. Waardenburg syndrome.
E. Williams syndrome

B  Horner Syndrome


Conventional antipsychotic medications (such as haloperidol, chlorpromazine, pericyazine) are used for the treatment of psychosis, anxiety and aggression in children and adolescents. One of the side effects of these medications is an increase in prolactin levels resulting in galactorrhea and amenorrhea in females and gynaecomastia in males.
Which of the following best describes the mechanism for this increase in prolactin?
A. Dopamine receptor agonist.
B. Dopamine receptor antagonist.
C. Increased gonadotropin-releasing hormone levels.
D. Serotonin receptor agonist.
E. Serotonin receptor antagonist

B  Dopamine receptor antagonist


Cystic fibrosis is an autosomal recessive disorder caused by mutations in a single gene on the long arm of chromosome 7. These mutations lead to abnormal functioning of the cystic fibrosis transmembrane conductance regulator (CFTR) protein, a chloride channel in the cell membrane. The most common mutation in the Australasian population is ∆F508.
What is the principal effect of this mutation on the function of CFTR?
A: Abnormal adenosine triphosphate (ATP) gating of the chloride channel.

B: Abnormal protein folding and thus poor transportation to the cell membrane.

C: Abnormal splicing resulting in reduced amounts of functional protein.

D: Decreased conductance of the chloride channel.

E: Premature transcription termination signals resulting in a truncated or absent protein.

B  Abnormal protein folding and thus poor transportation to the cell membrane


A 16-year-old girl is brought to the Emergency Department after attending a dance party. On presentation, she is extremely confused and agitated, she looks flushed and sweaty, and has dilated pupils. Her heart rate is 120 beats/minute and her blood pressure is 140/90 mmHg. She is complaining of some central chest pain.
Her presentation is most likely due to the ingestion of which of the following drugs?
A. Cannabis (Marijuana).
B. Diamorphine (Heroin).
C. Gamma Hydroxybutyrate (Grievous Bodily Harm, GHB).
D. Ketamine (Special K).
E. 3,4–Methylenedioxymethamphetamine (Ecstasy).

E 3,4–Methylenedioxymethamphetamine (Ecstasy)


Which of the following conditions is most likely to respond to selective serotonin reuptake inhibitors (SSRIs)?
A. Aggression.
B. Anxiety.
C. Autism.
D. Depression.
E. Tics.

B Anxiety


A 13-year-old boy presents with right upper quadrant pain and vomiting. He lives on a sheep station. On examination he is afebrile and has hepatomegaly.
An ultrasound scan confirms the presence of a 12 x 12 cm cyst, with several internal membranes, in the right lobe of the liver. His chest X-ray was normal.
Which of the following medical treatments is indicated?
A. Albendazole.
B. Doxycycline.
C. Metronidazole.
D. Pentamidine.
E. Praziquantel.

A Albendazole


Thyroid dysfunction due to which of the following is most likely to be missed on newborn screening?
A. Dyshormonogenesis.
B. Ectopic thyroid gland.
C. Panhypopituitarism.
D. Thyroid agenesis.
E. Transient hyperthyrotropinemia.

C Panhypopituitarism


The most significant change in auscultatory findings during a cyanotic spell in an infant with tetralogy of Fallot is that the:
A. ejection systolic murmur becomes louder.
B. ejection systolic murmur becomes softer.
C. pansystolic murmur becomes louder.
D. pansystolic murmur becomes softer.
E. second heart sound becomes more widely split.

B ejection systolic murmur becomes softer.


This family presents due to an apparent familial predisposition to a disorder


What is the most likely explanation for the apparent normal health of III:3?

A: Anticipation.
B: Imprinting.
C: Non-paternity.
D: Non-penetrance. X-linked inheritance

D Non-penetrance. X-linked inheritance


At 24-months of age, what percentage of children will spontaneously feed a doll with a pretend bottle?
A. 10%
B. 25%
C. 50%
D. 75%
E. 90%

E: 90%


An 11-year-old child undergoes muscle biopsy under general anaesthetic for a suspected myopathy. During the procedure she develops a high temperature and generalised stiffness. Biochemical testing shows acidosis, hypercapnia and a markedly elevated serum creatine kinase (CK).
The most likely diagnosis is:
A. halothane allergy.
B. malignant hyperthermia.
C. mitochondrial encephalomyopathy with lactic acidosis and stroke like episodes (MELAS).
D. neuroleptic malignant syndrome.
E. sepsis.

B. Malignant Hyperthermia


The initial luminal digestion of dietary starch by salivary and pancreatic amylase leads to the formation of which disaccharide?
A. Fructose.
B. Galactose.
C. Glucose.
D. Maltose.
E. Sucrose.

D Maltose


A ten-year-old girl is brought into the Emergency Department after being bitten by an unidentified snake. The snake venom detection kit is positive for brown snake venom from a bite site swab. Which of the following is the most common clinical effect in this envenomation?
A. Cardiotoxicity.
B. Coagulopathy.
C. Nephrotoxicity.
D. Neurotoxicity.
E. Rhabdomyolysis.

B Coagulopathy


A three-year-old girl presents following a hypoglycaemic seizure and has some investigations. Urinary metabolic screen confirms the diagnosis of Medium-Chain Acyl-CoA dehydrogenase deficiency (MCAD). The defect in this condition is most likely to cause hypoglycaemia by which of the following mechanisms?
A. Hyperinsulinism.
B. Impaired gluconeogenesis.
C. Impaired glycogenolysis.
D. Increased glycogen synthesis.
E. Increased ketone production.

B  Impaired gluconeogenesis


Which one of the following is the major determinant of risk of perinatal transmission of hepatitis C virus?
A. Breastfeeding.
B. Concomitant hepatitis B infection.
C. Concomitant human immunodeficiency virus (HIV) infection.
D. Maternal viral load.
E. Mode of delivery.

D Maternal viral load


Which of the following is the main site of potassium reabsorption in the nephron?
A. Proximal convoluted tubule.
B. Descending limb of the Loop of Henle.
C. Ascending limb of the Loop of Henle.
D. Distal convoluted tubule.
E. Collecting duct.

A Proximal convoluted tubule


The mechanism of action of inhaled nitric oxide is:
A. degradation of phosphodiesterase PDE5.
B. improved binding of oxygen to haemoglobin (Hb).
C. stimulation of β2 receptors.
D. stimulation of nitric oxide synthase.
E. upregulation of cyclic guanosine monophosphate (cGMP)

E: upregulation of cyclic guanosine monophosphate (cGMP)


Centre for Disease Control (CDC) body mass index (BMI) charts have been recommended as a screening tool to identify individuals who are overweight.

Which of the following BMI’s define a child likely to be obese?

A. >25 kg/m2

B. >30 kg/m2

C. >85th percentile for age

D. >85 <95 percentile for age.

E. ≥95th percentile for age.

E ≥95th percentile for age


A term infant with a history of neonatal sepsis was noted to have developed an area of subcutaneous fat necrosis over the left spiral groove of the humerus. She has a left-sided wrist drop as shown in the photograph below. Her deep tendon reflexes are normal

What nerve is most likely affected?
A. Axillary.
B. Median.
C. Musculocutaneous.
D. Radial.
E. Ulnar.

D Radial


What is the key developmental task of early infancy?

A: Autonomy.
B: Motor control.
C: Secure attachment.
D: Self-regulation.

E: Separation.

C Secure attachment


Which of the following coagulation factors is most likely to be relatively decreased in the healthy newborn compared to older infants?
A. factor V.
B. factor VII.
C. factor VIII.
D. Fibrinogen.
E. von Willebrand factor

B factor VII


A three-year-old boy is evaluated because of delayed motor milestones. On examination he has muscle weakness with calf hypertrophy. He has a markedly elevated creatine kinase level of 15,400 I/U [<240 I/U]. A diagnosis of Duchenne muscular dystrophy (DMD) is suspected and blood is taken for dystrophin gene testing.
Which of the following is most likely to be found on testing of the dystrophin gene?
A. Deletion of one base pair causing a frameshift.
B. Deletion of several exons.
C. Duplication of several exons.
D. Missense mutation.
E. Splice-site mutation.

B Deletion of several exons


Infliximab is effective in the treatment of Crohn’s disease. Its mode of action involves:
A. binding to tumour necrosis factor.
B. blockade of tumour necrosis factor receptors.
C. blockade of tumour necrosis factor secretion from inflammatory cells.
D. enhancement of hepatic metabolism of tumour necrosis factor.
E. inhibition of tumour necrosis factor production

A. binding to tumor necrosis factor


A patient in the Emergency Department has the laboratory results shown below:

Which of the following is the most likely cause of the acidosis?
A. Acute renal failure.
B. Diabetic ketoacidosis.
C. Lactic acidosis.
D. Renal tubular acidosis.
E. Salicylate poisoning

D. Renal tubular acidosis


Phototherapy is commonly used in the management of unconjugated hyperbilirubinaemia. The predominant mechanism of action of phototherapy is:
A. decreased binding of bilirubin to albumin.
B. destabilisation of photoisomers by bile acids.
C. geometric photo-isomerisation of unconjugated bilirubin to more soluble isomers.
D. intramolecular cyclisation to form lumirubin.
E. oxidation of bilirubin to form biliverdin and dypyrroles allowing excretion.

C. geometric photo-isomerisation of unconjugated bilirubin to more soluble isomers


Which medication has the best evidence for the treatment of adolescent depression?
A. Citalopram.
B. Fluoxetine.
C. Imipramine.
D. Paroxetine.
E. Venlafaxine.

B. Fluoxetine


A six-month-old child undergoes cardiac catheterisation. The oxygen saturations obtained in room air during the procedure are as follows:

These findings are most consistent with a diagnosis of:
A. atrial septal defect.
B. atrioventricular septal defect.
C. patent ductus arteriosus.
D. tetralogy of Fallot.
E. ventricular septal defect.

E. ventricular septal defect


Which serological finding in patients with hepatitis B indicates active viral replication?
A. Anti-HBc positive.
B. Anti-HBe positive.
C. Anti-HBs positive
D. HBeAg positive.
E. HBsAg positive

D. HBeAg positive


The incidence of autosomal recessive kidney disease in the population is 1:20,000. The population carrier frequency for the PKHD1 gene that leads to this disorder is closest to:
A. 1:50
B. 1:70
C. 1:100
D. 1:140
E. 1:400

B. 1:70


A ten-year-old boy with severe bronchiolitis obliterans presents to the Emergency Department with increasing exercise intolerance over the previous week.

A capillary blood gas shows the following picture:

This result is most consistent with which of the following?
A. Acute on chronic respiratory acidosis.
B. Acute respiratory acidosis.
C. Chronic respiratory acidosis.
D. Mixed metabolic and respiratory acidosis.
E. Uncompensated metabolic acidosis.

A. Acute on chronic respiratory acidosis


In acute paracetamol overdose, which of the following mechanisms may result in hepatotoxicity?
A. Conversion by cytochrome P450.
B. Conversion to glutathione conjugate.
C. Conversion to sulphate conjugate.
D. Direct toxic effect of unchanged drug.
E. Metabolism to phosphate form

A. Conversion by cytochrome P450


Which of the following provides the strongest stimulus for postnatal closure of the ductus arteriosus in a term infant?
A. Decreased circulating prostaglandin levels.
B. Decreased pulmonary vascular resistance.
C. Increased pulmonary blood flow.
D. Increased systemic oxygen saturation.
E. Sympathetic stimulation

D.  Increased systemic oxygen saturation


A 15-month-old boy recently completed chemotherapy for rhabdomyosarcoma. On routine testing the following results were obtained:

The drug most likely to have caused these abnormalities is:
A. cyclophosphamide.
B. dactinomycin.
C. etoposide.
D. ifosfamide.
E. vincristine

D. ifosfamide


In the treatment of chronic hepatitis C infection, which of the following is the most common side effect of ribavirin treatment?
A. Drug induced lupus.
B. Haemolytic anaemia.
C. Haemorrhagic cystitis.
D. Myocarditis.
E. Pancytopenia.

B. Haemolytic anaemia 


Which type of intervention is most likely to help children with delayed acquisition of reading skills?
A. Cerebellar exercises.
B. Combined phonics and whole word teaching.
C. Occupational therapy.
D. Stimulant medication.
E. Visual tracking exercises

B. Combined phonics and whole word teaching


A six-year-old girl presents after a fall from monkey bars. Her X-ray is shown below. On examination she has paraesthesia of her thumb, index and middle fingers, as well as an impalpable radial pulse.

Compression of which of the following neurovascular structures best explains the physical findings?
A. c
B. Brachial artery and radial nerve.
C. Brachial artery and ulnar nerve.
D. Radial artery and median nerve.
E. Ulnar artery and radial nerve

A. Brachial artery and median nerve.


Which of the following is not a mechanism used in the body for the production of endogenous glucose?
A. Activation of lipolysis.
B. Gluconeogenesis from lactate and alanine.
C. Glycogenolysis in the kidney.
D. Glycogenolysis in the liver.
E. Glycolysis in the muscle

E. Glycolysis in the muscle


Which of the following clotting factors is found in cryoprecipitate?
A. factor II.
B. factor V.
C. factor VII.
D. factor VIII.
E. factor X.

D. factor VIII


The following results were obtained from a randomised controlled trial of a new artificial surfactant called "S".


The number of babies that you would need to treat with “S” to prevent one case of chronic lung disease is:
A. 5
B. 10
C. 15
D. 20
E. 30

B. 10


A two-year-old boy presents with a six week history of diarrhoea and lethargy. Clinical examination reveals lower limb and periorbital oedema. Investigations show:

Normal liver function tests apart from albumin 22 g/L [35 - 50 g/L]. Urine analysis was normal.
Coagulation profile was normal.

What faecal test would confirm that the low albumin was related to gastrointestinal losses?
A. Alpha-1 antitrypsin.
B. Chymotrypsin.
C. Elastase.
D. Proalbumin.
E. Trypsin.

A. Alpha-1 antitrypsin


Boys with X-linked lymphoproliferative disorder are at highest risk of the development of severe illness or lymphoma following infection with which of the following pathogens?
A. Cytomegalovirus.
B. Epstein Barr virus.
C. Human herpesvirus 6.
D. Human herpesvirus 8.
E. Varicella zoster virus

B. Epstein Barr virus


Which of the following substances can induce expression of CYP3A4 and lead to a decrease in the blood level of cyclosporin?
A. Diltiazem.
B. Erythromycin.
C. Grapefruit juice.
D. Ketoconazole.
E. St John’s Wort

E. St John's Wort


Current extended newborn screening is most likely to miss disorders from which of the following groups?
A. Aminoacidopathies.
B. Disorders of vitamin B12 metabolism.
C. Fatty acid oxidation disorders.
D. Lysosomal storage disorders.
E. Organic acidaemias.

D. Lysosomal storage disorders


You are reviewing a 15-year-old boy with Duchenne muscular dystrophy (DMD) in your outpatient clinic. He has been in an electric wheelchair for three years. He has not required admission to hospital for more than five years

You receive the following results of his lung function testing:


Which of the following best describes these lung function results?
A. Mixed obstructive and restrictive pattern.
B. Moderate obstructive lung disease.
C. Moderate restrictive lung disease.
D. Poor technique.
E. Upper airway obstruction.

C. Moderate restrictive lung disease


Absorption of iron in the ferrous form (Fe 2+) occurs mainly in the:
A. stomach.
B. duodenum.
C. jejunum.
D. ileum.
E. colon

B. duodenum


A four-year-old boy presents with facial oedema and dark urine two weeks after a bout of tonsillitis. He has been anuric for 18 hours. Initial investigations confirm acute renal failure with markedly elevated serum urea and creatinine. His serum potassium is elevated at 6.8 mmol/L.
Which of the following reduces total body potassium?
A. Calcium infusion.
B. Glucose and insulin infusion.
C. Nebulised salbutamol.
D. Sodium bicarbonate infusion.
E. Sodium polystyrene sulphonate (Resonium) enema.

E.  Sodium polystyrene sulphonate (Resonium) enema.


The arterial blood pressure trace of an unwell one-year-old child is shown below.


The child has a one week history of low grade fever with viral symptoms. At presentation the child is tachycardic and tachypnoeic with poor peripheral perfusion. The most likely diagnosis is:
A. cardiac tamponade.
B. coarctation of aorta.
C. dilated cardiomyopathy.
D. septic shock.
E. viral myocarditis

A. cardiac tamponade


Which of the following is the most common adverse effect of carbamazepine (Tegretol)?
A. Alopecia.
B. Pancreatitis.
C. Rash.
D. Visual field restriction.
E. Weight gain.

C. Rash


Which of the following is the most useful test for assessment of disease activity in systemic lupus erythematosus?
A. Anti-nuclear antibody (ANA).
B. Complement factor 3 (C3).
C. C-reactive protein (CRP).
D. Extractable nuclear antigen antibodies (ENA).
E. Haemoglobin (Hb).

B. Complement factor 3 (C3)


Ultraviolet (UV) radiation (sunlight) acts on the skin to catalyse which of the following reactions?
A. 7-dehydrocholesterol to vitamin D3 (cholecalciferol).
B. 25-hydroxyvitamin-D to 1,25-dihydroxyvitamin D.
C. Cholesterol to 7-dehydrocholesterol.
D. Vitamin D2 (ergocalciferol) to vitamin D3 (cholecalciferol).
E. Vitamin D3 (cholecalciferol) to 25-hydroxyvitamin D.

A. 7-dehydrocholesterol to vitamin D3 (cholecalciferol)


What is the approximate half-life of serum immunoglobulin M (IgM)?

A. 1 day.
B. 7 days.
C. 21 days.
D. 3 months.

E. 6 months

B. 7 days


In infants with glucose-galactose malabsorption, ingestion of foods containing which of the following carbohydrates is likely to cause severe diarrhoea and dehydration?
A. Fructose.
B. Mannitol.
C. Mannose.
D. Sorbitol.
E. Sucrose

E. Sucrose


Which of the following observations regarding a rare disorder would provide the best evidence of autosomal dominant inheritance?
A. Father and daughter affected.
B. Father and son affected.
C. Male and female cousins affected.
D. Mother and daughter affected.
E. Mother and son affected

B. Father and son affected


A six-year-old boy is receiving induction chemotherapy for newly diagnosed acute lymphoblastic leukaemia. He is brought to the Emergency Department by his parents three days after receiving chemotherapy. He complains of an aching jaw and abdominal pain, and his parents report that he has not had a bowel action for three days.
The drug most likely to have caused these symptoms is:
A. asparaginase.
B. daunorubicin.
C. methotrexate.
D. prednisolone.
E. vincristine

E. vincristine


Ondansetron and granisetron are widely used as antiemetics post anaesthesia and in chemotherapeutic regimens. What is the mode of action of these drugs?
A. Anticholinergic.
B. Antihistamine.
C. Dopamine agonist.
D. Dopamine antagonist.
E. Serotonin antagonist

E. Serotonin anatgonist


Phenobarbitone is commonly used as the first line anticonvulsant in the treatment of neonatal seizures. In what percentage of cases would you expect to achieve clinical control of seizures after a loading dose of 20 mg/kg phenobarbitone?
A. 20%
B. 40%
C. 60%
D. 80%
E. 95%

B. 40%


Which of the following lipid-lowering drugs works primarily by the upregulation of low density lipoprotein (LDL) receptor activity?
A. Cholestyramine.
B. Clofibrate.
C. Colestipol.
D. Nicotinic acid.
E. Simvastatin

E. Simvastatin


A 3200 g term baby girl, born by ventouse extraction, is seen at five hours of age with recurrent apnoeic episodes. On examination she is afebrile, pale but well perfused, heart rate is 150 beats/min and mean arterial pressure is 42 mmHg. A computed tomography (CT) scan of her head is shown below

The scan is most suggestive of haemorrhage that is both intracerebral and:
A. extradural.
B. intraventricular.
C. subarachnoid.
D. subdural.
E. subgaleal.

C. subarachnoid


Which of the following psychoactive medications is most likely to cause excessive weight gain?
A. Fluoxetine.
B. Haloperidol.
C. Olanzapine.
D. Quetiapine.
E. Sodium valproate

C. Olanzapine


Human Metapneumovirus infection is associated with which of the following?
A. Bronchiolitis.
B. Croup.
C. Febrile convulsions.
D. Gastroenteritis.
E. Viral exanthem

A. Bronchiolitis


A three-year-old girl is diagnosed with adrenocortical carcinoma. Her maternal aunt died from osteosarcoma at 12 years of age, and her maternal grandmother was treated for breast carcinoma in her early forties. A familial tumour predisposition syndrome is suspected.
The most likely mechanism for this is a germline mutation in which of the following genes?
B. NF1.
C. p53.
D. RB1.

C. p53


Macrolide antibiotics have been increasingly used in the treatment of children with cystic fibrosis. The beneficial effect is most likely:
A. killing of atypical mycobacteria.
B. killing of Staphylococcus aureus
C. modulation of airway inflammation.
D. reduced adherence of Pseudomonas aeruginosa to airway epithelium.
E. upregulation of CFTR

C. modulation of airway inflammation


Which immunoglobulin in human breast milk is the most immunoprotective?
A. IgA.
B. IgD.
C. IgE.
D. IgG.
E. IgM

A. IgA


As glomerular filtration rate (GFR) declines, urinary creatinine clearance overestimates GFR because creatinine is:
A. metabolised by the tubules.
B. poorly filtered at the glomerulus.
C. reabsorbed by the tubules.
D. secreted by the tubules.
E. synthesized by the tubules.

D. secreted by the tubules