2011 B Remembered Flashcards
Parvovirus B19 has a number of clinical associations. Which of the following is NOT associated with Parvovirus B19?
A. Erythema Infectiosum
B. Hydrops foetalis
C. Myocarditis
D. Aplastic crisis in association with chronic haemolytic disorders
E. Transient erythroblastopaenia of childhood
E. Transient erythroblastopaenia of childhood
Erythema Infectiousum, fifth disease. Most common manifestation, average incubation 16d. Mild prodrome with ‘slapped-cheek’ phase1, then rash spreads to diffuse macular erythema (phase2) and resolves over 1-3w with waxing and waning.
Hydrops foetalis occurs secondary to profound foetal high-ouput cardiac failure from anaemia
Myocarditis Yes but rare. Foetal myocardium expresses P antigen, hence so dangerous in utero.
Aplastic crisis - absolutely. Think: sickle, thalassaemia, HS and pyruvate kinase deficiency. They tend to get a more severe sickness (unlike EI, not that unwell). Also, the incubation period is shorter in these patients.
Transient Erythroblastopaenia of childhood - Gaon: “TEC is a self-limiting and presents slightly later than Diamond-Blackfan anaemia. There are no fetal characteristics of the red cells. The children are phenotypically normal. It may be preceded by a viral illness such as human parvovirus infection”.
Also - arthropathy
Severe transient hypolastic anaemia
- 6/12 – 3 yo
- usually after 12/12
- cause unclear
- sometimes difficult to differentiate from Diamond-Blackfan syndrome
- normal HbF
- normal ADA
- 60% have associated neutropaenia
- develops slowly
- most kids recover within 1 – 2/12
- transfuse as necessary
An 8-week-old baby presents with prolonged jaundice and pale stools. Investigations reveal the following:
- Bilirubin 240 [0 – 50]
- Conj bilirubin 180 [0 – 10]
- GGT 320 [0 – 42]
- ALT 240 [9 – 40]
- ALP 250 [30 – 120]
Abdominal ultrasound revealed a contracted gallbladder.
Hepatobiliary iminodiacetic acid (HIDA) scan was non-excretory.
- PI type was ZZ
- CMV serology was IgG positive
- IgM negative
- Hepatitis A, B and C serology were all negative.
What is the most likely cause of this presentation?
A. a-1 antitrypsin deficiency
B. Biliary atresia
C. Choledocal cyst
D. CMV hepatitis
E. Tyrosinaemia
A. a-1 antitrypsin deficiency
PiZZ means can’t excrete the A1AT
B – this question doesn’t talk about status from birth, is this acute? LFTs are not cholestatic
C – would be seen on US, usually painful (RUQ)
D – CMV IgG is from mum, IgM –ve.
E – see below
Hepatitis serology all normal.
Remember, it’s a lung disease (A1AT is a protease inhibitor, protecting the lung from destruction by neutrophil elastase - but the A1AT accumulates in the liver, leading to metabolic disease).
Pi– (null) will give lung, but no liver disease. PiZZ = jaundice, pale stool and hepatomegaly in the first week of life, outcomes variable.
- Liver damage most commonly presenting as neonatal hepatitis with cholestasis beginning between four days and four months after birth and persisting for up to 12 months.
- Other clinical presentations among newborns included hepatomegaly with elevated aminotransferase levels (but without hyperbilirubinemia), ascites, and bleeding (often umbilical, superficial, or intracranial [5 percent of affected newborns]).
UTD:
- “HIDA scan is suggestive of BA but does NOT exclude other disease”
- PIZZ “results in defective secretion of protein X from hepatocytes”
presumably the build up of protein can cause the change seen on the HIDA. Remember that this result on the HIDA just means that the radioactive tracer is not seen in the gallbladder and so there is cystic duct obstruction of some sort….
Tyrosinaemia:
- Build up of tyrosine because unable to metabolise excess tyrosine
- Deficiency in FAH enzyme
- Affects liver, peripheral nerves and kidneys (Fanconi-like syndrome)
- Usually starts as an acute hepatic crisis, precipitated by acute infection
- Should have ++coagulopathy
An 8-year-old girl presents to the Emergency Department with her first generalised seizure. She has a past history of recurrent urinary tract infections. She has been otherwise well. On history, she has had 24 hours of generalised abdominal pain, headache and vomiting.
On arrival in the ED she is irritable, and complaining of altered vision. On examination she is afebrile, has a blood pressure of 158 / 80, and has blurred optic disc margins consistent with papilloedema.
An MRI of her brain is performed, and reveals a posterior encephalopathy as below. Immediately after the MRI she has a further generalised seizure, which is terminated
with IV midazolam. After the seizure her blood pressure is 178 / 104.
What is the most appropriate next step in medical management of this child?
A. Captopril
B. Frusemide
C. Mannitol
D. Nifedipine
E. Sodium nitroprusside
E. Sodium nitroprusside
This sounds like PRES (also known as RPLS). Posterior Reversible Encephalopathy Syndrome, secondary to malignant hypertension, which is this patient’s case may be from ARF/PN. Associated with eclampsia, Cyclosporin and Cisplatin.
IV Sodium nitroprusside best choice to decrease BP because of ability to titrate.
Nicardipine and labetalol are usually first line due to theoretical concern that nitroprusside may paradoxically increase intracranial pressure through vasodilation.
From UpToDate:
- Hypertension is a feature in the majority of PRES patients, regardless of aetiology.
- Significant improvement in pts with BP management.
- Usually only moderate HT in PRES, unless malignant hypertension.
- The initial aim of treatment in malignant hypertension is to rapidly lower the diastolic pressure to about 100 to 105 mmHg; this goal should be achieved within two to six hours, with the maximum initial fall in BP not exceeding 25 percent of the present level.
- Lowering to quickly may reduce the blood pressure below the autoregulatory range, possibly leading to ischemic events (such as stroke or coronary disease)
- *Mannitol** can worsen cerebral oedema
- *Nifedipine** is oral , can be sublingual. No longer used in emergencies because cannot control drop in BP.
A 10-year-old girl with a history of shortness of breath is brought to you for assessment.
Her lung fuction tests are as below.
- FVC 2.93L (83% predicted)
- FEV1 2.09L (67% predicted)
- FEV1/ FVC 71%
- PEF 5.9L/min 102%
Which best describes her results?
A. Poor technique
B. Mild obstructive disease
C. Mild restrictive disease
D. Mixed obstructive/restrictive disease
E. Normal lung function
?A
What is the primary reason for short stature in patients with Turner Syndrome?
A. Primary hypothyroidism
B. Growth hormone deficiency
C. X-inactivation (lyonisation)
D. Haploinsufficiency of the X-chromosome genes
E. Oestrogen deficiency secondary to ovarian failure
D. Haploinsufficiency of the X-chromosome genes
Almost all individuals with Turner syndrome have short stature. This is partially due to the loss of one copy of the SHOX (short-stature homeobox) gene on the X-chromosome. This particular gene is important for long bone growth.
Final adult height in Turner syndrome can be increased by a several inches if growth hormone (GH) treatment is given relatively early in childhood.
Which of the following problems with language development is characteristic of
children with Asperger syndrome?
A. Articulation difficulties
B. Expressive language delay
C. Pragmatic language difficulties
D. Receptive language delay
E. Voice production difficulties
C. Pragmatic language difficulties
A 9 month old Chinese girl presents with an eleven day history of fever, cough, extreme irritability and diarrhoea despite treatment with amoxicillin and regular paracetamol. Her immunisations are up to date. Bacille Calmette-Guérin (BCG) vaccine had been given at birth.
On examination she has mild conjunctivitis, red lips, circumoral pallor, a strawberry tongue and pharyngitis.
There is no significant lymphadenopathy. There is a faint erythematous maculopapular rash on her trunk and her BCG scar is acutely inflamed with redness and induration.
Blood tests from admission include:
The most likely diagnosis in this case is:
A. adenovirus infection.
B. enterovirus infection.
C. Kawasaki disease.
D. measles.
E. scarlet fever.
C. Kawasaki disease.
- Kawasaki – BCG induration. Specific sign
- Diarrhoea in 30%
Extreme irritability is a red-flag for KD. Should have thrombocytosis (increased platelets).
Support for KD: prolonged fever, rash, conjunctivae, oropharynx, irritable, BCG induration (specific to KD) raised ESR and WCC.
KD - fever plus 4/5: CODRaL (conjunctivae, oropharynx, desquamation, rash, lymphadenopathy)
The circumoral pallor makes you think Scarlet fever, but her fever should have gone by d7.
Not behaving like adenovirus – should it last this long?
Doesn’t sound like measles, should have lymphadenopathy and surely Koplick spots.
Based purely on the duration, induration of BCG site and irritability (plus satisfies criteria), ANS = D
Which of the following medications is most successful in treating social and generalized anxiety disorders?
A. Atomoxetine
B. Risperidone
C. Paroxetine (SSRI)
D. Imipramine
E. Sertraline (SSRI)
C. Paroxetine (SSRI)
Paroxetine - anxiety
Fluoxetine – depression
Social Anxiety Disorder (SAD), first line and most successful = SSRI
Note: Atomoxetine is an NRI (selective NRI), not an SNRI (serot/NA reuptake inhibitor – Venlafax).
You are asked to assess a 12 hour old boy that was born at 35/40. The labor was 24 hours long and the mother received 4L of 5% Dextrose and Normal saline, as well as a syntocin infusion during that period. The baby was born via Ventouse and APGARS were 3 and 6.
A blood glucose level was checked at 4 hours of age, which was 1.4mmol/L. The baby commenced 80mL/kg/day of intravenous 10% Dextrose.
You are asked to review the baby 5 hours later because it is jittery. You repeat blood
tests:
- BSL 3.0
- Na 130
- K 5.6
- Cl Normal
The hyponatremia in this instance is most likely due to:
A . Too much IV Fluid given to the mother during labour
B. Too much IV Fluid given to the baby since birth
C. Renal Na losses
D. Adrenal dysfunction due to prematurity
E. Inappropriate ADH secretion due to mild HIE
A . Too much IV Fluid given to the mother during labour
UptoDate says that in the early newborn period, hyponatremia, defined as a serum sodium concentration of 128 or less, most often reflects excess total body water with normal total body sodium. This may result from increased maternal free water intake or the syndrome of inappropriate antidiuretic hormone secretion (SIADH). SIADH may accompany pneumonia or meningitis, pneumothorax, or severe intraventricular hemorrhage.
Hyperglycaemia not an issue, so unlikely dilutional pseudohyponatraemia. It is reasonable to give a prem 80mkd, so exclude B.
Valproate increases side effects of Lamotrigine via which mechanism?
A. Protein binding
B. Induction of enzyme
C. Competitive inhibition of enzyme
D. Renal clearance
E. Conjugation
C. Competitive inhibition of enzyme
Valproate inceases SEs of Lamotrigine and lamotrigine increases SES of Carbamazipine (VLC)
A four-year-old girl has aortic stenosis. She is admitted with fever, and a new murmur is noted.
Prior to initiating management for presumed infective endocarditis, which of the following investigations must be performed?
A. Three blood cultures over one hour.
B. Three blood cultures over 24 hours.
C. Three blood cultures with fevers.
D. Tranoesophageal echocardiogram.
E. Transthoracic echocardiogram.
F. Three blood cultures at one hour intervals
B. Three blood cultures over 24 hours.
From UTD: In the critically ill child, three separate venipunctures for blood cultures should be performed as quickly as possible (less than one hour), and empiric antibiotic therapy started. If the patient is not acutely ill, antibiotic therapy can be withheld for 24 to 48 hours while the blood cultures are collected.
….in this question there is no mention that the child is acutely unwell.
Echo can be done at any time.
Current guidelines for IE prophylaxis…
The 2008 Australian guidelines recommend antibiotic prophylaxis only for patients with the following cardiac conditions which are thought to be associated with the highest risk of adverse outcomes from endocarditis if undergoing a specified dental or medical procedure:
- Prosthetic cardiac valve or prosthetic material used for cardiac valve repair
- Previous infective endocarditis
- Cardiac transplantation with the subsequent development of cardiac valvulopathy
- Congenital heart disease but only if it involves:
- Unrepaired cyanotic defects, including palliative shunts and conduits
- Completely repaired defects with prosthetic material or devices whether placed by surgery or catheter intervention, during the first 6 months after the procedure (after which the prosthetic material is likely to have been endothelialised)
- Repaired defects with residual defects at or adjacent to the site of a prosthetic patch or device (which inhibit endothelialisation
Antibiotic choice….
Dental procedures - oral amoxicillin 2g given 30 to 60 minutes before the procedure.
…..not to be confused with BICILLIN given IM in RHD…..
An eight-month-old girl presents with fever for 18 hours. On examination, she appears well and there is no obvious focus of infection. Her immunizations are up to date.
Which of the following organisms is most likely to be positive on blood culture?
A. E. Coli.
B. Neisseria Meningitidis.
C. Staph. Aureus.
D. Strep. Pneumoniae.
E. Listeria monocytogenes.
D. Strep. Pneumoniae.
(even fully immunised, still most common)
At 8m, she has finished her courses of DTP, IPV, PnC and rotaV. Almost finished her hepB and HiB. Not yet had her MMR and menC, nor VZV.
A mother brings her 18 month old boy in for review because he has a unilateral testicular mass that has been enlarging over 3 weeks. The boy has no evidence of
virilisation. Ultrasound shows the mass to be solid. Alpha-fetoprotein level 1700 (N<7).
(picture shows unilateral testicular mass – no overlying skin changes)
The mass is most likely:
A. Germinoma
B. Gonadoblastoma
C. Teratoma
D. Leydig cell tumour
E. Yolk sac tumour
E. Yolk sac tumour
Alpha feto protein in 18 m old most likely Yolk sac – will produce AFP and sometime BHG
- Gonadoblastoma – less likely
- Teratoma – solid, mixed components, tooth, hair
- NOT leydig cell, not write age and not these markers
- Germ cell tumour – from yolk sac of embryo
- Pure germinoma – often don’t produce tumour cell markers like BHCG
Epithelial lining – 1 of very very few carcinoma type in children
Emedicine – most common germ cell tumours are teratoma or yolk sac tumour.
Alpha feta protein = yolk sac tumour
BHCG = hepatoblastoma
Germinoma 10% high HCG
You are asked to see a four-month-old in the Intensive Care Unit. He has been hypotonic and weak since birth. On examination, there is no facial weakness but he has profound weakness of the extremities with areflexia. His chest X-ray is shown.
The most likely diagnosis is:
A. Congenital myotonic dystrophy.
B. Mitochondrial myopathy.
C. Nemaline myopathy.
D. Pompe disease.
E. Spinal muscular atrophy.
D. Pompe disease.
A 3 month old girl presented in severe DKA that initially required an insulin infusion and IV fluids. She is now on subcutaneous insulin therapy. Her genotye comes back and shows she has a kir 6.2 mutation. What management change should you institute?
A. Cease all insulin
B. Commence insulin pump
C. Commence metformin
D. Commence a sulfonylurea
E. Continue her current subcutaneous insulin
D. Commence a sulfonylurea
Sulphonylurea – kir 6.2 is one of the mutations associated with neonatal diabetes and sometimes maturity onset diabetes of the young (MODY) or Monogenic diabetes. Commonly these conditions respond to Sulphonylurea’s that act specifically on the channels involved in insulin secretions that are affected by the genetic problems. Kir 6.2 specifically is discuss in UTD and is best treated with a sulphonylurea.
A four year old boy has this ECG performed as part of the work up for a suspected seizure.
What does it show?
A. Left atrial hypertrophy
B. Left ventricular hypertrophy
C. Normal ECG
D. Right bundle block
E. Right ventricular hypertrophy
E. RVH
According to Park, criteria for RVH are
- Right axis deviation,
- Tall R-waves in V1 & V2,
- Abnormally large R/S ratio in V1/V2 and small R/S ratio in V6,
- Upright T-waves in V1 >3 days (other sources suggest between 7 days and 7 yrs)
A 33 week baby boy is born at 1700g. His apgar scores are 5 and 7 at 1 and 5 minutes respectively. He has some respiratory distress and an initial oxygen requirement of 28%. He is commenced on intravenous benzylpenicillin and gentamicin. After 12 hours his respiratory distress has resolved and antibiotics are ceased. He no longer requires oxygen.
On day 2 he develops jaundice with a total bilirubin of 200 μmol/L and a conjugated bilirubin of 19 μmol/L. He receives 24 hours of phototherapy, after which his total bilirubin is 98 μmol/L.
At 2 weeks he develops apnoeas, thought to be reflux-related. His feeds are thickened. There is a maternal uncle in the extended family with a history of hearing loss.
At two months, this baby has a hearing test, which reveals profound hearing loss. Which of the following is most likely to have contributed to his hearing loss?
A. Apnoea.
B. Family history.
C. Gentamicin.
D. Jaundice.
E. Low birth weight.
B. Family history.
50% of sensorineural hearing loss is hereditary, with 1/3 being syndromic and 2/3 non-syndromic. Most commonly AR so distant family history is to be expected, but is often not identified at all.
A prospective randomised control trial is conducted with a group of frequent relapsing nephrotic syndrome patients. Group A is treated with cyclosporin with the dose adjusted for trough levels, where as Group B are treated with a consistent dose of cyclosporin.
The results showed the hazard ratio for relapse was 0.43 (95% CI of 0.17 – 1.09) Group A compared to Group B. What is the best interpretation of this result?
A. Group A are 43% less likely to relapse than Group B
B. Group A are 57% less likely to relapse than Group B
C. Group A have 43% the rate of relapse of that in Group B
D. Group A have 57% the rate of relapse of that in Group B
E. Group A had significantly less relapses that Group B
C. Group A have 43% the rate of relapse of that in Group B
Hazard ratio means – TAKES TIME INTO ACCOUNT, time to an event or survival ratio between things
A seven-year-old boy with cerebral palsy presents with 36 hours of fever, cough and increasing respiratory distress. He has vomited twice. On examination his temperature is 39.2 degrees celcius and he has bronchial breathing and coarse crackles in the right upper zone, with moderate work of breathing. His chest x-ray is shown:
What is the most appropriate choice of intravenous antibiotic?
A. Benzylpenicillin.
B. Benzylpenicillin and Roxithromycin.
C. Cefotaxime.
D. Cefotaxime and Metronidazole.
E. Flucloxacillin and Gentamicin.
A. Benzylpenicillin.
E. Flucloxacillin and Gentamicin.
Depends whether the real XR in the exam showed a cavitating lung lesion (as here) or aspiration (as per the stem).
- Cover for aspiration - just use penicillin for aspiration pneumonia
- Infection above diaphragm = penicillin and below - metronidazole
- If caviting lesion with air fluid level – use flucox and gent
The test, Thiopurine s-methyltransferase (TPMT), is used for guiding therapy with which of the following drugs?
A. Methotrexate
B. Azathioprine
C. Mycophenolate
D. Cyclosporin
E. Cyclophosphamide
B. Azathioprine
TPMT is an enzyme involved with metabolism of AZA and it’s metabolites and low enzyme activity can result in higher levels of toxic metabolites with increased risk of myelosuppression or liver toxicity.
A 15 year old boy presents with unilateral right sided breast swelling for 3 months. He has been well with no discharge from the breast. On examination he is well grown, weight and height on the 90th centile. He is pubertal at stage 3 pubic hair and genital development with 15cc testes bilaterally. His right breast is enlarged and mildly tender. His left breast is normal.
Which is the most likely diagnosis?
A. Breast abscess
B. Breast cancer
C. Prolactinoma
D. Klinefelters
E. Pubertal gynecomastia
E. Pubertal gynecomastia
Breast abscess will usually be unwell with significant pain and likely purulent discharge and is very rare in this age group. Breast cancer is extremely rare. Prolactinoma would be bilateral and cause gallactorrhoea, Klinefelters almost always have <5mL testes
Which condition is most associated with an increased intensity in the pulmonary component of the 2nd heart sound?
A. ASD
B. Pulmonary hypertension
C. Severe pulmonary stenosis
D. Tetralogy of Fallot
E. Transposition of Great Arteries
B. Pulmonary hypertension
7 year old girl presents following a first episode of a brief seizure involving her right arm. Her family emigrated from India to Australia 2 years ago. She and her family are vegetarians. She has been well with no fevers, no headaches and no ear aches. Her physical examination, including neurological examination is normal.
Her MRI is shown below:
(image similar to that shown BUT showing occipital lesion, report given below)
MRI of brain with gadolinium contrast on T2 weighting shows cystic lesions and nodules with slight ring enhancement present in the right temporal and subcortical regions. There is a further cystic lesion in the right occipital region with marked peri- lesional oedema.
Which of the following is the most likely diagnosis?
A. Brain abscess
B. Hydatid disease
C. Neurocysticercosis
D. Trichinosis
E. Tuberculoma
C. Neurocysticercosis
Classic tape worm – usually solitary lesion, India, 2 years (takes long time – latent period), oedema causes seizures. Most common cause of seizured in the world. Treatment: albendazole but can make them worse so MUST use steroid cover to redeuce swelling. Some say don’t treat us the parasite is dying anyway.
Usually transmitted via pork, but can be fecal-oral via humans.
Brain abscess would commonly have one or more of headache, fever and focal neurology. Hydatid is rarely outside of the liver and lung. Trichinosis is either GI or severe widespread. Tuberculoma follows systemic TB infection and if anything causes focal neurology and signs of mass effect.
Secrets: tapeworm disease, commonly from ingestion of undercooked pork. If haematogenous spread of the eggs to the brain occurs, cysts form that can result in seizures/other neurological manifestations. MRI: ring enhancing cysts characteristic of the disease
Which has been shown to most effectively reduce the rate of suicide?
A. Cognitive behavior therapy
B. Media blackout on reporting suicide
C. Restricted access to lethal means
D. Media campaigns
E. Family therapy
C. Restricted access to lethal means
Children with Beckwith-Wiedermann syndrome are at risk of developing Wilms tumours, therefore regular renal ultrasound scanning should be done.
What is the cumulative risk in BWS of a Wilms tumour?
A. 1%
B. 5%
C. 15%
D. 25%
E. 50%
B. 5%
A 3 year old boy presents with a one day history of fever, coryza and cough. On examination he looks unwell is mildly hypoxic in room air and is tachypnoeic. Urinalysis reveals Blood ++ and Protein +. Creatinine is mildly elevated (~90), other bloods normal. During your assessment, he coughs up bright red blood. Coagulation studies are normal.
What test is likely to confirm the diagnosis?
A. Angiotensin converting enzyme (ACE)
B. ANCA
C. ANA
D. Quantiferon gold release assay
E. C3
B. ANCA
ANCA positive small vessel vasculitis includes Wegener’s and can classically present with pulmonary-renal syndrome. i.e. C-ANCA
Wegener’s: a necrotising granulomatous vasculitis of the upper and lower respiratory tracts, accompanied by GN. Clinical features: pulmonary granuloma, destructive granuloma of the ears, nose and sinuses, rash, GN, eye lesions. Ix with ANCA. Rx with steroids and cyclophosphamide.
ACE Is for Sarcoid, very rare. ANA is for lupus which is not as rare but very rarely presents this way. Quantiferon for TB might present with haemoptysis (although this is more an adult presentation) but unlikely nephritis. Low C3 in SLE, PSGN, MPGN but these won’t present like this.
ANCA-associated vasculitis:
- Wegener’s = granulomas + respiratory + kidneys, F>M - cANCA
- Microscopic polyangiitis = similar to WG but not granulomatous, M=F - pANCA
- Churg-Strauss syndrome = small vessel granulomas + refractory asthma + eosinophilia, M=F, usually adults – pANCA
- p-ANCA = perinuclear. Target = myeloperoxidase
- c-ANCA = cytoplasmic. Target = proteinase 3
A 16 year old primigravida gives birth to a 2.5kg term baby girl. There is a history of poor antenatal care, prolonged rupture of membranes and a maternal fever. Fragile skin and blistering is noted to be present at birth which worsens with handling.
(picture of a baby with skin blistering on torsoe and fingers. Illustrative photo shown)
What is the most likely diagnosis?
A. Epidermolysis Bullosa
B. Group B Streptococccal infection
C. Herpes Simplex Virus
D. Pemphigus Vulgaris
E. Staphylococcal Scalded Skin Syndrome
A - Epidermolysis Bullosa
Epidermolysis bullosa (EB) comprises group of rare inherited disorders characterized by marked mechanical fragility of epithelial tissues, with blistering and erosions following minor trauma.
It is the only one that can present from birth (UTD). SSSS is a key differential but usually presents a few days down the line.
Among newly arrived refugee children, what percentage are affected by anxiety, depression and/or post-traumatic stress disorder?
A. 5%
B. 10%
C. 20%
D. 30%
E. 50%
E. 50%
You are seeing a 2 year-old boy who has recently been discharged after his 5th admission with viral wheeze associated with an upper-respiratory tract infection. On each occasion he has responded well to asthma treatment and been discharged within 48 hours. His parents report that there have been no exercise- or allergen- induced interval symptoms of wheeze, difficulty breathing or cough. The boy has not suffered from eczema and there is no family history of atopy though his father reports having similar episodes as a young child that he “grew out of”.
His parents are concerned and would like to avoid further episodes and admissions if possible.
What is the most appropriate advice at this time?
A. Fluticasone 100mcg BD
B. Fluticasone 750mcg BD during upper respiratory tract infections
C. Oral prednisolone 1mg/kg at the first sign of wheeze
D. Inhaled salbutamol 6 puffs 4-hourly at the first sign of wheeze
E. Salmeterol 50mcg BD during upper respiratory tract infections
B. Fluticasone 750mcg BD during upper respiratory tract infections
**Intermittent inhaled glucocorticoids **
- Intermittent use, started at the onset of an URTI and continued for approximately one week may decrease asthma-type symptoms and oral glucocorticoid use in preschool children with viral-associated wheezing.
- Fluticasone 750 mcg BD for 7-10 days at first sign on URTI, before wheeze onset recommended by UTD.
Inhaled short-acting beta agonists
- first line in virus-induced wheezing and are an effective rescue treatment in symptomatic patients, especially in children with established asthma.
- not been shown to improve clinical outcomes, decrease the rate of hospital admission, or decrease the duration of hospitalization in children with bronchiolitis.
Mother presents with her child having toddler tantrums. What is the best way to manage these?
A. Physical punishment (eg. smack)
B. Incentive system (eg. Star Chart)
C. Stop, Think, Do
D. Time Out
E. Verbal Reprimand
D. Time out
- Identify tantrum triggers. Certain situations – shopping, visiting or mealtimes – might frequently involve temper tantrums. Think of ways to make these events easier on your child. For example, you could time the situations so your child isn’t tired, eats before you go out, or doesn’t need to behave for too long.
- When a tantrum occurs, stay calm (or pretend to!). If you get angry, it will make the situation worse and harder for both of you. If you need to speak at all, keep your voice calm and level, and act deliberately and slowly.
- Wait out the tantrum. Ignore the behaviour until it stops. Once a temper tantrum is in full swing, it’s too late for reasoning or distraction. Your child won’t be in the mood to listen. You also run the risk of teaching your child that tantrums get your full involvement and attention.
- Make sure there’s no pay-off for the tantrum. If the tantrum occurs because your child doesn’t want to do something (such as get out of the bath), gently insist that she does (pick her up out of the bath). If the tantrum occurs because your child wants something, don’t give her what she wants.
- Be consistent and calm in your approach. If you sometimes give your child what he wants when he tantrums and sometimes don’t, the problem could become worse.
- Reward good behaviour. Enthusiastically praise your child when she manages frustration well.
A 9 month old girl presents with a fever and lethargy. She is diagnosed with an E. Coli urinary tract infection following investigation and is treated with 48 hours of IV ampicillin and gentamicin. There is no family history of vesico-ureteric reflux and she had normal antenatal ultrasounds.
What would be the most appropriate next investigation?
A. DMSA in 4-6 months
B. Ultrasound and DMSA in 4-6 months
C. Ultrasound and MCU in 4-6 weeks
D. Ultrasound in 6 weeks
E. Ultrasound prior to discharge
D. Ultrasound in 6 weeks
I think this question is remember wrong because being nine months this child actually requires no imaging according to the guidelines and that’s not one of the answer options…
<6 months
For an atypical or recurrent UTI, USS during acute infection.
For a typical UTI which responds to antibiotics within 48 hours - USS within 6 weeks, if USS abnormal consider MCUG.
DMSA at 4-6 months, and MCUG if atypical or recurrent UTI.
6m-3yrs
For an antypical UTI, USS during acute infection.
For recurrent UTI - USS within 6 weeks.
DMSA at 4-6 months if atypical or recurrent UTI.
No MCUG
A 10-year old girl with known Type 1 diabetes mellitus is found to have an anti-tissue transglutaminase level of 120 (0-5) following routine screening for coeliac disease. She is referred to a gastroenterologist who performs a gastroscopy. The histology from multiple small bowel biopsies shows no evidence of coeliac disease.
What is the next most appropriate step in management of this girl?
A. Repeat the anti-tTG
B. Repeat the gastroscopy in 12 months
C. Start a gluten-free diet
D. Do HLA DQ2/DQ8 typing
E. Screen the family for coeliac disease
B. Repeat the gastroscopy in 12 months
The following diagram represents a family tree of individuals with Leber hereditary optic neuropathy. What is the probability that the person indicated will be affected?
A. <5%.
B. 25%.
C. 50%.
D. 80%.
E. 100%.
E. 100%
Leber hereditary optic neuropathy is mitochondrially inherited but, due to mitochondrial heteroplasmy, there are some children of an affected mum who don’t inherit it and, because there is also variable penetrance, some children who do inherit it may not actually have symptoms.
Furthermore, it would depend on whether the questions asked about the condition being ‘inherited’ or the child actually being ‘affected’.
A 3-year-old boy has chronic mucocutaneous candidiasis. Which of the following conditions is he most at risk of developing?
A. Diabetes mellitus
B. Hypothyroidism
C. Disseminated fungal infection
D. Coeliac disease
E. Systemic Lupus Erythematosis
A. Diabetes mellitus or
B. Hypothyroidism
Chronic mucocutaneous candidiasis (CMCC) is a heterogeneous group of syndromes with common features including chronic noninvasive Candida infections of the skin, nails, and mucous membranes and associated autoimmune manifestations (most commonly endocrinopathies).
Mutations in the AIRE gene cause CMCC and when associated with endocrine abnormalities is called autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED).
The classic triad is mucocutaneous candidiasis, hypoparathyroidism, and adrenal failure
Other endocrinopathies associated with include:
- Type 1 diabetes mellitus
- hypothyroidism
- growth hormone deficiency
- ovarian failure (appears to coexist with adrenal failure)
- male hypogonadism.
A 15-year old girl presents to the emergency department requesting emergency contraception 48 hours after having unprotected intercourse.
To be effective, emergency hormonal contraception should be administered within
A. 24 hours
B. 36 hours
C. 48 hours
D. 72 hours
E. 96 hours
D. 72 hours
A one day old baby is cyanotic and tachypnoeic. First and second heart sounds are normal and there is a 3/6 pansystolic murmur loudest at the lower left sternal edge. There is evidence of hepatomegaly. The chest xray is shown below:
What is the most likely diagnosis?
A. Dextro-transposition of the great arteries
B. Persistent fetal circulation
C. Ebstein anomaly
D. Tricuspid atresia
E. Tetralogy of Fallot

C. Ebstein anomaly
- Atrialisation of RV
- tricuspid regurgitation and right-sided cardiac chamber dilation and dysfunction.
- Cyanosis due to LA to RA flow through ASD because PA may be obstructed by leaflet of Tricuspid valve
- ECG finding include
- RAH + RBBB
- Low voltage QRS over right sided chest leads
- CXR - In severe cases, the chest radiograph reveals massive cardiomegaly (often termed a “wall to wall” heart) with diminished pulmonary vascularity. The right atrium is prominent and the left heart border becomes straight or convex due to dilated and displaced right ventricular outflow. The chest radiograph may be normal in patients with less severe disease.
A 14-year-old vegan girl presents with severe tiredness. Three weeks previously she was treated for fever, cough and dyspnoea with a seven-day course of amoxycillin.
Laboratory tests include:
Blood film: polychromasia, microspherocytes, rouleaux, agglutination.
Which of the following is the most likely underlying cause of this anaemia?
A. Hereditary spherocytosis.
B. Infectious mononucleosis.
C. Mycoplasma pneumoniae infection.
D. Penicillin-induced haemolysis.
E. Vitamin B12 deficiency.
C. Mycoplasma pneumoniae infection.
Cold agglutinin disease.
A 12yo boy is diagnosed with inflammatory bowel disease and commenced on corticosteroids. His symptoms progress until he develops fever and bloody stools. Examination shows a slightly distended abdomen with generalised tenderness. He has the following AXR taken.
(the image was a poor quality, but similar to this one.
There were arrows pointing to a dilated gas-filled loop of small bowel in the LUQ.)
What does the x-ray show?
A. Perforation
B. Small bowel obstruction
C. Toxic megacolon
D. Large bowel obstruction
E. Volvulus
C. Toxic megacolon
Toxic megacolon can occur as a complication of Shigella infection, pseudomembranous colitis, Hirschsprung disease, or inflammatory bowel disease.
UTD:
CLINICAL MANIFESTATIONS — Toxic megacolon affects all ages and both sexes. Patients with IBD are at the highest risk for developing this complication early in their disease, not infrequently at initial presentation.
Signs and symptoms of acute colitis that are frequently resistant to therapy are often present for at least one week prior to the onset of acute dilatation. Severe bloody diarrhea is the most common presenting symptom, while improvement of diarrhea may herald the onset of megacolon. One study of patients with C. difficile colitis and toxic megacolon found that diarrhea was a complaint in 100 percent of cases, malaise in 91 percent, and abdominal pain and distention in 82 percent.
A 15 year old female who has spent some time in Malaysia in the past year, presents with haemoptysis. She has a two month history of fatigue and weight loss. Her x ray is shown below.
Her sputum show 3+ for acid fast bacilli and she is diagnosed with tuberculosis. You are advised that sensitivities from her sputum culture will take approximately 4-6 weeks.
Which of the following antibiotic regimens is most appropriate?
A. Isoniazid
B. Isoniazid and Pyrazinamide
C. Isoniazid and Rifampicin
D. Isoniazid, Rifampicin, Amikacin and Moxifloxacin
E. Isoniazid, Rifampicin, Pyrazinamide and Ethambutol
F. Isoniazid and Pyridoxine
G. Isoniazid, Rifampicin, Myambutol and Ethambutol
E. Isoniazid, Rifampicin, Pyrazinamide and Ethambutol (RIPE)
This regimen 1st line
2 months 4 drugs, then 4 months 2 drugs (isonazid and rifampicin)
This girl has active TB and is highly infectious. Needs 2 months of RIPE then another 4 months (6m total) of isoniazid and rifampicin.
A 3 year old boy has a two week history of rash that initially began as a pruritic red papular rash on his hands and feet. In the last week his GP commenced him on a course of cephalexin.
The eruptions have now evolved further (pictured below) :
What is the most likely diagnosis:
A. Eczema herpeticum
B. Infected flea bites
C. Hand foot and mouth diseases
D. Infected Scabies
E. Drug Eruption
D. Infected scabies
Classic distribution
Treatment = permethrim. 1 dose and then another in 2/52
Image shows burrows which are pathognomonic of scabies.
A 3 month old boy presents with a recent history of coryzal symptoms including cough and rhinorrhoea. His oxygen saturations are 90% in room air and he is noticeably tachypnoeic. His CXR is as shown below :
What is the most likely diagnosis?
A. ASD
B. Tetralogy of Fallot
C. Transposition of Great arteries
D. Ebstein anomaly
E. AVSD
B. Tetralogy of Fallot
Boot shaped heart, normal heart size, oligaemic lungs, borderline sats, clear lung-fields (note that a hypoplastic main pulmonary artery contributes to the ‘boot’ appearance).
Characteristic CXR findings in CHD:
- Boot-shaped heart = Tetralogy = concave main PA segment with upturned apex
- Egg-shaped heart = TGA
- Snowman sign = supracardiac TAPVR
- Rib-notching = coarctation (seen in older children)
- Massive cardiomegaly = Ebsteins (or Pompe)
Tetralogy of Fallot:
- 5-10% of all CHD – the most common cyanotic heart defect
- RVOT obstruction (usually infundibular stenosis) + large perimembranous VSD, from which RVH and over-riding aorta follow
- Presentation: Murmur audible from birth (PS). Most are cyanotic at birth or shortly thereafter (acyanotic may be asymptomatic or develop signs of CHF from large L-R shunt)
- ECG: RAD (normal axis if acyanotic TOF) + RVH (BVH in acyanotic form)
- CXR: boot shaped heart as above (acyanotic TOF can look like a moderate VSD but also have RVH compared to VSD alone which normally shows LVH)
A baby is born at 36 weeks with IUGR. Weight was on the 10th centile, Length 10th centile and head circumference 2nd centile. The child has increased respiratory effort and a photo is shown (has a blueberry muffin rash).
The most likely cause is:
A. CMV
B. Toxoplasmosis
C. Syphilis
D. Rubella
E. Parvovirus
A 2 year old girl presents after a prolonged seizure in the setting of an URTI. She has a background of mild developmental delay and behavioral problems. On examination she looks well but has increased tone and reflexes and positive babinski reflex.
An MRI is shown below:
What is the diagnosis?
A. Adrenoleukodystrophy.
B. ADEM
C. Krabbe disease
D. Metachromatic leukodystrophy.
E. HHV6 encephalitis.
A photograph of an 18 month old girl is shown below.

What is the most common associated heart defect?
A. ASD.
B. Tetralogy of Fallot
C. Pulmonary stenosis.
D. Coarction of the aorta.
E. Hypertrophic cardiomyopathy.
A 3 year old presents after a brief period of unresponsiveness. He is pale and sweaty and is pulse is difficult to identify. He is also hypotensive.
His ECG demonstrates is shown:
The most appropriate treatment is:
A. Vagal manouvre.
B. Intravenous adenosine.
C. Intravenous beta blocker.
D. Unsynchronised direct current (DC) shock.
E. Synchronised direct current (DC) shock.
(A newborn chest X Ray was shown with a chest drain in the right upper zone. Lung markings were visible in the right upper, but not lower zone. The mediastinum was not displaced.)
The newborn baby was retrieved from a peripheral hospital with tachypnoea and respiratory distress. He was diagnosed with a pneumothorax and a chest drain was inserted as shown. Following chest drain, he became more tachypnoeic, tachycardic and developed even further increased work of breathing.
The most likely diagnosis is:
A. Blocked Chest tube
B. CCAM
C. Lobar Emphysema
D. Bronchogenic cyst
E. Tension pneumothorax
2 year old boy presented with fever, pancytopenia, hepato-splenomegaly, elevated ferritin, LDH, ESR and CRP. Which virus is the most likely trigger for this disease.
A. Adenovirus
B. Parvovirus
C. EBV
D. HIV
E. HSV
F. Human herpes virus 6
(The histology diagram is similar to above, showing haemophagocytosis)
A girl presents with right-sided facial weakness and crusting of the right ear as shown in the picture.
What other finding is most likely on examination:
A. Diploplia
B. Right Abducens weakness
C. Sparing of the forehead muscles on the right side
D. Loss of hearing on the right side
E. Loss of taste on the posterior third of the tongue.
A 2 year old boy was brought to the Emergency Department with a history of being unwell for the last 3 days. He and was febrile, tachycardic and tachypnoec. A chest x-ray and a 12 lead ECG was performed and is provided below:
What is the most likely diagnosis?
A. ALCAPA
B. Myocardial infarction
C. Pericarditis
D. Pulmonary embolism
A 5 year old boy presents with poor sleep and breathing problems since his first attack of acute tonsillitis six months ago. His parents report that he snores very loudly and sometimes stops breathing in the middle of the night. They also complain that he is very sleepy and tired during the day despite having almost 11 hours of sleep every night.
Examination of his throat reveals the following (similar picture in exam)
What is the best form of management?
A. Adenotonsillectomy
B. Continuous positive airway pressure (CPAP) at night
C. Oral steroids
D. Oral antibiotics
E. Intranasal corticosteroids
