2010 PART B
Q1) A 4 year old girl presents with recurrent bacterial UTIs, requiringIV antibiotics on 2 occasions. There is no history of constipation. Her pants are always wet. There is no history of urgency or holding behaviours.
Ultrasound shows left sided hydronephrosis and a dilated left collecting system.
Picture of L) anterior pyelogram showing dilated pelvis and “filling defect” / ureteric systole at proximal ureter, and rest of ureter dilated plugging into bladder.
What is the most likely diagnosis?
A)Ectopic ureter
B)Neuropathic bladder
C)PUJ obstruction
D)VUJ obstruction
E)VUR
Q2)A child presents with a Wilm’s tumour, with favourable histology. CT of the abdomen (pictured) showeda Wilm’s tumour in the right kidney. CT chest showed lung metastases.
What is the cure rate if he is treated with standard therapy?
A)35%
B)50%
C)65%
D)90%
E)95%
Q3)A 4 year old child presents with bulky greasy stools, chronic diarrhoea, and weight loss. Neurological examination demonstrates lower limb hyporeflexia and gait exam elicits ataxia. Which vitamin is he deficient in?
A)Vitamin A
B)Vitamin B12
C)Vitamin C
D)Vitamin D
E)Vitamin E
Q4) (repeat 2008 and 2009)
A6 year old boy presents with asthma and is currently taking Flixotide 125ug BD, with daytime cough and wheeze. He is using his Ventolin 10-12 x per day.
[RFTs given and show an obstructive loop
There is a 92% increase in FEV1post bronchodilator]
What is the most appropriate next step in this patient’s management?
A)Start alternate day steroids
B)Increase Flixotide to 250 mcg x2 BD
C)Add monteleukast
D)Add salmeterol 5mcg BD
E)Change to oral budesonide 100 x2 BD
Q5)A 3 year old girl presents withintermittent swellingof the hands, feet and face. These episodes occur every few weeks. Her parents note that they began aroundthe time they moved house one year ago. She has an allergy to peanuts.
What is the most likely diagnosis?
A)C1 esterase inhibitor deficiency
B)congenital lymphoedema
C)Idiopathic chronic urticaria
D)IgE mediated nut allergy
E)IgE medicated mould allergy
Q6) A 10 year old boy has sensorineural hearing loss and short stature. Both his height and weight are on the third centile. He needs assistance at school with reading and maths. He is noted to be more fidgety than usual. On examination he has a smooth goitre
TFTs -FT3 and FT4 elevated, TSH 4.0 (upper limit given was 4.0)
What is the most likely diagnosis?
A)Graves Disease
B)Pendred Syndrome
C)Subacute thyroiditis
D)Ectopic TSH production
E)TSH receptor defect
Q7) a 4 year old boy awoke with severe calf pain. He is unable to walk. He is keeping his feet plantarflexed and complains of pain and strongly resists your dorsiflexion. On examination his reflexes are present except for the ankle jerks are unable to be illicited.
What is the most likely diagnosis?
A)Viral myositis
B)Dermatomyositis
C)Guillian Barre syndrome
D)Transverse myelitis
E)Epidural abscess
Q8)The most common side effects of stimulants used to treat ADHD is:
A)Anxiety
B)Appetite suppression
C)Drowsiness
D)Lack of spontaneity
E)Motor tics
Q9) A 4yo child undergoes a Bone Marrow Transplant. 14 days later he develops tender hepatosplenomegaly, weight gain, ascites and mild hyperbilirubinaemia.
The most likely diagnosis is:
A)Budd-Chiari syndrome
B)Congestive cardiac failure
C)CMV hepatitis
D)Graft vs Host Disease
E)Veno-occlusive disease
Q10) A 32/40 infant is born with congestive cardiac failure. An ECGis done (pictured) - shows ?widespread ST elevation and complete heart block.
The most likely diagnosis is:
A)complete heart block
B)hyperkalaemia
C)hypocalcaemia
D)MI
E)Torsade
OR
Q10) A Neonatal ECG A neonate is noted to be bradycardic and in heart failure with liver edge to 4cm. An ECG is done(pictured) -shows narrow QRS in lead II but very elevated and depressed st segments in all other leads, looking like wide QRS. P waves not in relation to QRS complexes.
What is the most likely diagnosis?
A)Myocardial infarction
B)Complete (3rd degree) heart block
C)Pericarditis
Q11) A 15yo girl presents with secondary amenorrhoea. She is obese, has acne, facial hirsutism, acanthosis nigricans, and is found to be hypertensive at 130/80.
Her blood test results are as follows.
Glucose 5
Insulin 25 paired
17-OH-progesterone 5 high normal
LH/FSH 3
What is the most appropriate next step in her management?
A)GnRH analogue
B)Metformin
C)OCP
D)suppression of adrenal steroids
E)diet and exercise
Q12) An 18 month old was found by the parents in bed several times with moaning episodes.Each lastedfor as long as half an hour, andthey occur 2-3 hours after going to sleep. She is unresponsive during theepisodes,and there wasassociated sweating and pupil dilatation.
The most likely diagnosis is:
A)Confusional arousals
B)Nocturnal frontal lobe epilepsy
C)Night mares
D)Sandifer syndrome
E)Night terrors
Q13) A 7 yr old presents with abdominal pain. There is also a history of lethargy, anorexia and weight loss. Bilateral medullary nephrosis is noted on investigation. Abdominal X-ray is shown (shows bilateral renal calculi)
The renal ultrasound scan is otherwise normal.
The most likely diagnosis is:
A)Cystinuria
B)Idiopathic hypercalciuria
C)Distal RTA
D)Medullary sponge disease
E)???
Q14) A 15yr old previously treated for ALL now presents with bilateral ankle pain and swelling. A Lateral x-ray of feet is shown. (??Talar collapse on xray, and ??? osteopaenia).
The most likely cause for these xray findings is:
A)Asparaginase
B)Ciprofloxacin
C)Dexamethasone
D)Vincristine
E)Cyclophosphamide
Q15) A 14 year old boy with a history of focal segmental glomerular sclerosis is 14 days post deceased donor transplant and presents with heavyproteinuria.
What is the most likely cause of this presentation?
A)Thrombosis in graft
B)BK virus
C)Graft versus host disease
D)Humoral rejection
E)CMV
Q16) A 15 year old girl has a few brief seizures and is found unconscious. She has empty packets of tramadol, paracetamol, amitryptilline and (something else?ibuprofen) beside her.Her GCS is 10. Her heart rate and blood pressure are stable. Her ECG is below (shows inverted T waves in all leads, wide QRS). Electrolytes are in the normal range
Which of the following is the first line treatment for her ECG changes?
A)Adrenaline
B)insulin and glucose
C)Salbutamol
D)Sodium bicarbonate
E)Flumazenil
Q17) A female infant who has features of Turner syndrome is noted to have an ejection systolic murmur.
What is the most likely diagnosis?
A)ASD
B)Bicuspid aortic valve
C)Coarcation of the aorta
D)Peripheral pulmonary stenosis
E)VSD
Q18) A previously well 2 year old girl presents with 24 hours of abdominal pain associated with 12 hours of vomiting. The last two vomits have had flecks of blood in them. Her vital signs are normal.
What is the most likely diagnosis?
A)H. pylori infection
B)Mallory-Weiss tear
C)Oesophageal varices
D)Oesophagitis
E)Peptic ulcer
Q19) A term infant presents at 16 hours of age with apnea. On examination the infant is noted to be tachypneic with saturations in air of 70%, good volume pulses, a normal S1 and a loud and single S2. The ECG is normal and the CXR shows a slight increase in heart size.
What is the most likely diagnosis?
A)Atrioventricular septal defect
B)Hypoplastic left heart syndrome
C)Tetralogy of Fallot
D)Transposition of the great arteries
E)Truncus arteriosus
Q20) A 5 year old girl undergoing induction chemotherapy for acute lymphoblastic leukaemia develops mucositis and odynophagia. Candida glabrata is cultured from her mouth.
The most suitable agent is:
A)Caspofungin
B)Itraconazole
C)Ketoconazole
D)Miconazole
E)Terbinafine
Q21) (Repeat 2009)
A 6 month-old boy with recurrent fevers requires hospital admission.Which sign or symptom is more likely to be seen in cyclical neutropenia rather than periodic fever with aphthous ulcers, pharyngitis and adenitis syndrome (PFAPA)?
A)Abdominal pain
B)Diarrhoea
C)Folliculitis
D)Gingivitis
E)Tonsillar hypertrophy
Q22) A female infant was born at term weighing 3450g. Apgars were 8 and 9 at 1 min and 5 mins respectively. Over the first week she feeds poorly and does not regain her birthweight.An MRI scan shows absent/abnormal signal from the basal ganglia with sparing of the thalamus and posterior limbs of the internal capsule.
[An MRI shown but report given in question]
Which of the following is most likely to cause the findings?
A)Arterial Ischaemic stroke
B)Congenital Toxoplasmosis
C)GBS Meningitis
D)Hypoxic Ischaemic Encephalopathy
E)Sinovenous thrombosis
Q23) The extended Newborn Screening Program testing in Australia and New Zealand using Tandem Mass Spectrometry can detect an increased range of conditions. Which of the following CANNOT be detected using this method?
A)Amino acid disorders
B)Fatty Acid oxidation disorders
C)Glycogen Storage Disorders
D)Maternal B12 deficiency
E)Organic Acidaemias
Q24) A 6yo male with a known diagnosis of Hyper IgE syndrome presents with cough and fever.
[CXR shown. ?R mid zone lateral opacity. Poor quality x-ray, couldn’t clearly see an air-fluid level or not]
Which of the following is the most likely organism?
A)Aspergillus fumigatus
B)Candida albicans
C)Klebsiella pneumoniae
D)Pneumocystis jiroveci
E)Staphylococcus aureus
Q25) A Term infant presents with coughing and cyanotic episodes during feeds.Birth history includes ruptured membranes for 1 hour, normal vaginal delivery with meconium liquor. The infant was initially good condition at birth with good apgars.It subsequently developed respiratory distress at a few hours of age, woth SaO2 91%, RR 70. A NGT was inserted and CXR performed.
(CXR showed NGT coiled in upper airway with large stomach bubble and ?R diffuse side hazy changes.)
What is the most likely cause of this presentation?
A)Aspiration pneumonia
B)Congential pneumonia
C)Hyaline membrane disease
D)Meconium aspiration syndrome
E)Transient tachypnoea of newborn
Q26) A 2 yo with diagnosis of cystic fibrosis presents with theirfirst respiratory infection with cough and loss of weight.
What is the most likely pathogen?
A)Aspergillus
B)Burkholderia cepacia
C)Haemophilis influenzae (non-typeable)
D)Pseudomonas
E)Staph aureus
Q27) A 5yo female returned from 6 week holiday in Malaysia 3 weeks ago. On return she had developed a non-itchy facial rash as shown.
(Photo shown of girl of asian descent with bilateral macular erythematous rash (?scales present) with sharp edges and sparing of nasolabial folds. Remainder of skin of face normal.)
What is the most likely cause of this presentation?
A)Adenoma sebaceum
B)Atopic dermatitis
C)Lupus
D)Scarlet fever
E)Slapped cheek
Q28) A 7 month Indian child who is fully breast fed has a lower limb XR as shown
(XR of lower limbs consistent with rickets - genu varum, decreased bony opacification, widened metaphysis of distal femur).
What is the likely cause in this child?
A)Dietary calcium deficiency
B)Dietary phosphate deficiency
C)Maternal vitamin D deficiency
D)Malabsorption of vitamin D
E)Decreased vitamin D metabolism
Q29) A 3 week old boy presents with his head tilted and turned towards the right and on examination he has a mass in the right sternocleidomastoid muscle.
What is the most likely cause?
A)Benign Paroxysmal Torticollis
B)Congenital Torticollis
C)????
D)Tumour
E)Positional Plagiocephaly
Q30)A 7yo with chronic granulomatous disease presents with a three week history of persistent dry cough. A CT Chest is performed and shows bilateral nodules.
(CT Slice given)
Most likely cause is?
A)Aspergillus
B)Candida
C)Mycobacterium
D)Staph Aureus
E)PCP (PJP)
Q31) A 2 year old Indian boy presents with pallor. Mother reports that he is otherwise well. His blood tests are as follows: (reference ranges given)
Hb 33
MCV 46
Retics 87
WCC and Diff - normal
Platelets– normal
The most likely cause of his pallor is:
A)Iron Deficiency
B)Beta Thalassaemia
C)Autoimmune Haemolysis
D)Aplastic Anaemia
E)Vit B12 Deficiency
Q32) What is the commonest malignancy that occurs after renal transplant?
A)CNS tumour
B)Leukaemia
C)Lymphoproliferative disease
Q33)(repeat 2008 Adult Paper 1 Question 26)
The figure below shows the adjusted relative risk from cardiovascular causes among women in United States cities at various levels of exposure to fine particulate matter (PM2.5). The cohort in the Women’s Health Initiative is analysed using the Cox proportional hazards model (New England Journal of Medicine 2007 Vol 356 (5))
Confidence limits (shown in gray) are widest at exposure levels above 24 ug/m3 because:
A)the association is strongest at very high exposure levels.
B)very few women have this level of exposure.
C)the association is not causal.
D)confounding is more likely in cities with high pollution levels.
E)the hazards are not proportional.
Q34) A10 yo boy presented with faecal soiling, which always happens at home but has never happened at school. His parents have recently separated. His physical examination is normal apart from a mass palpable at left lower quadrant. What is your initial management?
A)Psychotherapy
B)Family therapy
C)Laxatives
D)???
E)???
Q35) A woman has sensorineural hearing loss and also has long QT syndrome, with a past history of implantable cardiac defibrillator after syncope at age 4. Assuming she has a non-consanguinous marriage, what is the chance her child will inherit long QT?
A)<5%
B)25%
C)33%
D)50%
E)75%
Q36) A five year old boy presents with a 5 day history of swelling on the left lower eyelid. He is afebrile and the swelling is non-tender. He has a normal FBE and CRP of 5. What is the most likely diagnosis?
(Picture shows lump on left lower eyelid lateral margin, minimal overlying erythema but some yellowish discharge)
A)Chalazion
B)dermoid cyst
C)stye
D)sebaceous cyst
E)???
Q37) A fifteen year old girl presents with collapse and rhythmic jerking of limbs. She awakes normally with no confusion. She has no incontinence. This is the 12th similar episode in 3 months. An EEG is normal.
This is most consistent with:
A)complex partial convulsions
B)factious disorder
C)hypochondria
D)vasovagal syncope
E)???
Q38)(repeat 2008Bq100, 2009)
A male infant born at 26 weeks gestation, who is now four weeks old, is fully established on enteral milk feeds which are fortified to a total caloric intake of 150 kcal/kg/day. He had mild hyaline membrane disease but is now breathing in 23% supplemental oxygen, to maintain oxygen saturations between 90% and 94%. He is no longer having apnoeas and his only medication is a vitamin supplement. His birthweight plotted on the 50th percentile and is now plotting between the 3rd and 10th percentile for weight.
His blood results are as follows:
Sodium 134 mmol/L [135 – 145 mmol/L]
Potassium 4.7 mmol/L [3.5 – 5.1 mmol/L]
Chloride 98 mmol/L [98 – 110 mmol/L]
Bicarbonate (HCO3) 20 mmol/L [20 – 26 mmol/L]
Haemoglobin (Hb) 10.1 g/dL [13.0 – 18.0 g/dL]
The most likely cause for this infant’s inadequate weight gain is:
A)anaemia of prematurity.
B)high intestinal losses of chloride.
C)high renal losses of sodium.
D)inadequate caloric intake.
E)metabolic acidosis.
Q39) A 6 weeks old baby who is fully breastfed presents with with painless PR bleeding. The baby is well and thriving.
The most likely diagnosis is:
A)allergic proctitis
B)anal fissure
C)intussecption
D)Meckel's diverticulum
Q40)A 16 yr old primigravida mother with poor antenatal care gives birth. There is a history of maternal fever during labor. The baby born with peeling skin as shown in the picture. (photo of newborn term baby with peeling of skin at minimal handling)
The most likely responsible organism is:
A)Herpes simplex
B)Staph aureus
C)Group B streptococcus
D)?Epidermolysis bullosa
E)?Phemphygus vulgaris
Q41) A 4yo boy is brought to see you by his parents. They describe him as often 'vague'. He has been assessed by a speech pathologist who reports he has delayed expressive and receptive language delay. He has also been seen by a psychologist who reports that his development is otherwise normal. His hearing is normal. His parents report he is 'obsessed' with trains. When he does not get his way he gets angry and physically lashes out.
What developmental problem is he at highest risk of developing in his school years?
A)ADHD
B)High functioning autism
C)OCD
D)ODD
E)Reading disorder
Q42) A 14yo girl presents with primary amenorrhoea. She is on the 75th centile for height and the 50th centile for weight. She has stage 1 pubic hair, and stage 4 breast development. She has normal female external genitalia. An ultrasound reveals gonads in the inguinal canal, and no uterus.
Blood tests reveal:
Oestrogen < 20 (low)
FSH and LH high
Testosterone 20 (high)
What is the most likely diagnosis?
A)Gonadal failure
B)Complete AIS
C)Mosaic Turner syndrome
D)True hermaphrodite
E)Gonadal dysgenesis
Q43) A 6yo girl presents with 3 weeks of fever, a transient rash (worse when febrile), myalgia, malaise, and weight loss of 1kg. On examination she looks unwell, with evidence of recent loss of weight. She has a 2-3cm liver edge and a tippable spleen. Cardiovascular, respiratory, ENT, and joint examinations are unremarkable.
Blood tests reveal:
Hb 98
WCC 20
Pl 585
ESR 105
CRP 180
Ferritin 5000 (very high)
Triglycerides 1.2
What is the most likely diagnosis?
A)Acute Lymphoblastic Leukaemia
B)Kawasaki’s Disease
C)Macrophage Activation Syndrome
D)Rheumatic fever
E)Systemic onset JIA
Q44) A 4yo boy presents with cough and difficulty breathing. One week ago he went to the GP with tender inguinal lymphadenopathy and was commenced on flucloxacillin.On examination he is afebrile, with a BP of 132/88, and mild peripheral oedema. He is tachypnoeic with crackles on auscultation.
He has a CXR [normal heart size but wet lung fields, no focal consolidation]
Urinalysis reveals 3+ protein and 3+ protein [no blood results given]
The most appropriate initial treatment is:
A)Captopril
B)Frusemide
C)Hydralazine
D)Metoprolol
E)Nifedipine
Q45) (repeat 2009)
Which of the following is the most common side effect of steroid use in children?
A) Behavioural change
B) Hypertension
C) Decreased glucose tolerance
D) Decreased immunity during infection
E) Peptic ulceration
Q46) An infant born at 36 weeks is admitted to NICU for tachypnoea and poor feeding. He is cool peripherally and pale. He was born to a primigravida, whose membranes had ruptured 4 days prior to delivery. She developed a fever late in labour (no comment on IV antis in labour or GBS status) There was thick meconium present at delivery. His temperature is 37, other observations within normal limits. A Chest xray is shown(shows normal sized heart, not overexpanded, both left and right lungs have patchy changes, L>R.)
What is the likely cause of his respiratory distress?
A)Meconium aspiration
B)Pneumothorax