2009 remembered Flashcards
A 10 year old boy presents with poorly controlled asthma. He is taking 125 micrograms of fluticasone
twice daily and is using salbutamol up to 10 times a day. Spirometry demonstrates an obstructive
pattern with a 91% improvement following administration of salbutamol. Which of the following is
the next most appropriate management step?
A. Increase fluticasone to 250 micrograms twice daily
B. Add salmeterol
C. Add Montelukast
D. Commence prednisolone
E. Replace fluticasone with budesonide
B) salmeterol
SIGN guidelines: add a long acting beta agonist next
15 year old boy with a history of congenital heart disease presents with recurrent collapse. On
arrival is appears well with normal vital signs. He then collapses with no pulse palpable. An ECG
demonstrates a broad complex tachycardia. Which of the following is the next most appropriate
management?
A. Cardiac compressions
B. Asynchronous shock
C. Synchronous shock
D. IV adenosine
E. Intubate and ventilate
B. Asynchronous shock
“In a witness cardiac arrest with immediate identification of VF/pulseless VT, up to 3 stacked asynchronous shocks of 2J, 4J, 4J/kf may be given.”
A child presents with suspected Marfan syndrome. She is tall with arachnodactyly, has an arm span
greater than her height and has a known aortic root dilatation. Which of the following would be
most helpful in confirming the diagnosis?
A. Hypermobility score
B. Slit lamp examination
C. Pelvic x-ray
D. Lumbar spine x-ray
E. Scoliosis
B. Slit lamp examination
A child on treatment for acute lymphoblastic leukemia is found to be hyponatremic. His weight has
been stable for the past 5 days, and he feels otherwise well.
Bloods: Na 124, K 4.7, Cl 95, osmolality low
Urine: Na high, Cl high, K normal, osmolality high
Which of the following is the most likely diagnosis?
A. Iatrogenic – excessive intravenous hypotonic saline
B. SIADH
C. Diabetes insipidus
B. SIADH
Which of the following most differentiates cyclic neutropenia from Periodic Fever, Aphthous
stomatitis, Pharyngitis, and cervical Adenitis (PFAPA)?
A. Folliculitis
B. Gingivitis
C. Diarrhoea
D. Abdominal pain
B. Gingivitis
Haematologic disorder and form of congenital neutropenia that tends to occur approximately every three weeks and lasting for few days at a time due to changing rates of neutrophil production by the bone marrow. It causes a temporary condition with a low absolute neutrophil count and makes the body in severe risk to inflammation and infection.
A 14 year old girl, with a known history of SLE - treated with cyclophosphamide, presents with a 2
day history of a facial rash (picture of a rash on her face which is over the right ear, anterior to the
ear, and on the neck, erythematous with punched out lesions). The rash came on rapidly and was
painful.
Which of the following will be the most appropriate medication? A. Aciclovir B. Flucloxacillin C. Pimecrolimus D. Tetracycline E. Atretinoin
A. Aciclovir
Ramsay-Hunt Syndrome
- The major otologic complication of VZV reactivation is the Ramsay Hunt syndrome, also known as Herpes zoster oticus.
- It typically includes the triad of
o Ipsilateral facial paralysis
o Ear pain
o Vesicles in the auditory canal and auricle
- Taste perception, hearing (tinnitus, hyperacusis), and lacrimation are affected in selected patients.
- It is generally considered a polycranial neuropathy with frequent involvement of cranial nerves V, IX, and X.
- Vestibular disturbances (vertigo) are also frequently reported.
- Ramsay Hunt syndrome has also been reported in association with HSV-2 infection.
- Ramsay Hunt syndrome has been liked to reactivation of latent VZV residing within the geniculate ganlgion with subsequent spread of the inflammatory process to involve the VIII cranial nerve. This results in auditory and vestibular disorders.
- The facial paralysis seen in Ramsay Hunt syndrome is felt generally to be more severe than Bell’s palsy attributed to HSV, with increased rates of late neural denervation and a decreased probability of complete recovery.
Which of the following is the best indication for intervention to close an atrial septal defect in a 4
year old child?
A. To increase exercise tolerance
B. To decrease chest infections
C. To decrease the risk of endocarditis
D. To decrease the risk of stroke
E. To avoid pulmonary hypertension in future life
E. To avoid pulmonary hypertension in future life
Indications for closure of a large atrial septal defect
Isolated secundum ASDs <6 mm diameter in infants close spontaneously by two years, and some as late as 5 years – so no early closure
Moderate size (at least 6 to 8 mm in diameter) and larger are relatively unlikely to close spontaneously. However, small chance of spontaneous closure so no tx <2y
Isolated secundum ASDs with a large left-to-right shunt resulting in symptoms or significant right heart enlargement, usually associated with a ratio of pulmonary to systemic flow (Qp/Qs) exceeding 2:1. The AHA has recommended a threshold Qp/Qs ≥1.5:1, while the Canadian Cardiac Society recommended a threshold Qp/Qs >2:1, or >1.5:1 in the
presence of reversible pulmonary hypertension
Also: small ASD post embolic strokes or TIA (paradoxical embolus)
A 3 month old well baby presenting for routine review, is noted to have an abnormal shaped head.
Which of the following is the most likely diagnosis?
A. Cephalhematoma
B. Metopic suture synostosis
C. Coronal suture synostosis
D. Essential plagiocephaly
D ?
Order of most commonly prematurely fused sutures = sagittal, coronal, metopic and lambdoid.
Closure of Fontanelles Posterior – 2 months Anterior – 9-18 months Delayed Closure of the fontanelles Hydrocephalus, Hypothyroidism, Rickets, Malnutrition, Osteogenesis imperfect, Chromosomal abnormalities eg Tr21, Alpert syn
Sagittal Craniosynostosis - Scaphophcephaly – long and narrow skull
• Premature closure of the sagittal suture, the most common cause form of craniosynostosis
• long and narrow skull, prominent occiput, broad forehead
• increased A-P diameter
• small or absent anterior fontanelle
Coronal craniosynostosis - Frontal plagiocephaly
♀ > ♂
• fusion of either right or left side of coronal suture
• forehead and brow stop growing on that side
• contralateral prominent forehead
• elevation of ipsilateral orbit and eyebrow
Fusion of both coronal sutures is Brachycephaly
• increased bi-parietal diameter
• decreased A-P diameter
• often syndromic
Unilateral or bilateral mid and upper face hypoplasia may occur. Orbits may be eliiptical and the supraobrital ridge may not be formed well
Occipital/Lambdoid Craniosynostosis
• Occipital ‘plagiocephaly’ is rarely due to fusion of sutures
• Fusion of the lambdoid suture results in unilateral occipital flattening. The ear on the flattened side is posterior, has a bump behind it and sticks out more.
• Most often, occipital plagiocephaly is a result of head positioning in infancy or with an immobile infant/child
Metopic craniosynostosis - Trigonocephaly
Results in a narrow triangular forehead with pinching of the temples laterally
Prominent ridge running down forehead and gives appearance of hypotelorism
You are shown a picture of a boy with several café au lait macules on his trunk. He is also
macrocephalic, has short stature and has a mild learning disability. His yearly follow up should
include which of the following?
A. Spinal X-ray
B. EEG
C. Neuroimaging
D. Echocardiogram
E. Ophthalmological review and slit lamp examination
F. Renal ultrasound
E. Ophthalmological review and slit lamp examination
- Evaluate the child for new neurofibromas and progression of lesions. Examine the skin carefully for signs of plexiform neurofibromas that may impinge on or infiltrate underlying structures.
- Check the child’s blood pressure yearly to determine if there is evidence of hypertension, which occurs more frequently with NF1 and could be secondary to renal artery stenosis, aortic stenosis, and pheochromocytoma, the latter being more common in adults.
- Evaluate neurodevelopmental progress of an affected child.
- Obtain a formal ophthalmologic evaluation yearly.
- Evaluate the child for skeletal changes. Look for scoliosis, vertebral angulation, and limb abnormalities, particularly tibial dysplasia. Sometimes localized hypertrophy of a leg, arm, or other part of the body results from plexiform neurofibromata. Nonossifying fibromas of the long bones infrequently occur in adolescence or adulthood and have been associated with fracture; although a screening radiograph of the knees in adolescence has been suggested as a routine study, evidence is not sufficient to support routine screening at this time.
- If any unexplained complications occur or if cutaneous lesions appear to be growing rapidly, refer the patient to the appropriate subspecialist for further evaluation.
The 11 year old girl shown below presents with a weight > 97th centile, height < 3rd centile, a blood
pressure of 120 /85 and a decreased growth velocity. She has Tanner Stage 3 pubic hair, Stage 2
breast development and sparse axillary hair.
Which of the following tests is most likely to lead to the diagnosis?
A. 24 hr urinary cortisol
B. ACTH
C. Androstenedione
D. LH/FSH
E. Abdominal ultrasound
A) 24 hr urinary cortisol
A 12 year old boy presents with a 3 year history of intermittent diarrhoea/semi solid stools. He has
elevated transaminases, 1+ fat globules and 2+ fatty acids with no blood in his stool, a lipase of 600,
and ESR of 30 and a hypochromic microcytic anaemia (in words). Which of the following is the most
likely diagnosis?
A. Coeliac disease
B. Crohns disease
C. Colitis
D. Irritable bowel
E. Chronic pancreatitis
A. Coeliac disease
(alt q: normal Total IgA, elevated serume anti-gliadin IgA and IgG, Normal anti-ttg IgA
??E) chronic panc
A boy presents with mild eczema and a thrombocytopenia. Which of the following tests would best
distinguish between Wiskott Aldrich Syndrome and ITP?
A. IgG
B. Immunoglobulins
C. Platelet volume
D. Platelet antibodies
E. T and B cell numbers
C. Platelet volume
A 5 year old boy is found to have a visual field defect. An MRI Brain (pictured) demonstrates a
cerebellar/optic pathway tumour. Which of the following is the most likely associated syndrome?
A. NF1
B. NF2
C. Tuberous Sclerosis
D. Von Hippel Lindau Syndrome
E. Sturge Weber Syndrome
A. NF1
Eye signs in above conditions:
• NF-1: optic nerve gliomas in 15%, lisch nodules, choroidal naevi
• NF-2: early onset cataracts, retinal hamartomas, bilateral acoustic neuromas (?papilloedema)
• TS: retinal hamartomas, eyelid angiofibromas
• VHL: retinal capillary haemangiomas
• MS: optic neuritis
A mother presents to you with concerns about the rotavirus vaccine. She has heard that there was
an increased rate of intussusception with the vaccine in the US and she wants to know the best way
of preventing intussusception with the Rotateq vaccine. Which of the following has the best
evidence for preventing intussusception with rotateq?
A. Give the first dose before 3 months
B. Give the last dose after 6 months
C. Give separately to other vaccinations
D. Defer until after rotavirus season
E. Withhold until before winter
A. Give the first dose before 3 months
The greatest risk of intussusception occurred within 3 to 14 days after the first dose, with a smaller risk after the second dose.
There is evidence that when the first dose of RotaShield was given at >3 months of age, the risk of intussusception was increased.
A 7 year old boy presents with vocal and motor tics of 3 months duration. What can you tell his
mother about the natural history of his tics?
A. They will likely decrease in frequency over the next 3-4 months
B. They will continue and get worse with viral infections
C. They will wax and wane with improvement in puberty
D. They will improve over the next 6 months
E. They will resolve in 2-3 years time
C. They will wax and wane with improvement in puberty
In DSM-IV-TR, a tic is defined as a sudden, rapid, recurrent, nonrhythmic, stereotyped motor movement or vocalization that is experienced as irresistible, but can be suppressed for varying lengths of time. They are usually markedly diminished during sleep.
Tourette’s disorder (TD) or syndrome (TS) is characterized by multiple motor and one or more vocal tics that have been present at some time in the illness, although not necessarily concurrently. The tics occur many times a day nearly every day for more than 1 yr with no more than 3 consecutive tic-free months. Chronic motor or vocal tic disorder is similar, but each does not include both kinds of tics. Transient tic disorder involves motor and/or vocal tics that have been present for at least 4 wk, but less than 1 yr.
The onset of a tic disorder almost always occurs during childhood, developing in approximately 5-10% of early-school-aged children, with spontaneous disappearance in approximately 65% by the onset of adolescence.
Which of the following is required for the diagnosis of Tourettes’ syndrome?
A. Dystonia and tics for at least 6 months
B. Vocal and motor tics for at least 6 months
C. Vocal and motor tics for at least 12 months
D. Tics and OCD
C. Vocal and motor tics for at least 12 months
A 10 month old child has an 8 week history of being unwell with fever, anorexia and weight loss. He
now presents with crying for the past 24 hours and doesn’t want to be handled. On examination: HR
160, RR 44, Cap refill 4 seconds. Hb 85, WCC 38, Neutrophils 26, Plts 900, CRP 140, LDH high. Urine
is normal. A CT abdomen similar to below is shown.
Which of the following at is the most likely diagnosis? A. Wilm’s tumour B. Neuroblastoma C. Psoas abscess D. Appendiceal abscess E. Lymphoma
B. Neuroblastoma
Abdominal mass + fevers + weightloss + hip pathology + 8week duration – 10 months
?? C) psoas abscess
Prof Downie – Does not think this is neuroblastoma. Metastatic neuroblastoma into marrow. 10m usually stage 4s and not into marrow. If this sick would prob be in marrow but then WCC not high. Not presentation in this age group. Prob psoas abscess but he never seen it. Not Wilms, not lymphoma. Blood count not quite right for neuroblastoma. More likely infection with Crp and WCC
A 2 year old girl presents with a one week history of fever and cough. She has been on amoxicillin
with no improvement. Her respiratory rate is 60, and her temperature is 40C. On examination, she
has decreased breath sounds at the left base with dullness on percussion. A CXR is shown and
demonstrates an almost white out on the left side. Which of the following is the best choice of
treatment?
A. Azithromycin
B. Cefotaxime and flucloxicillin
C. Ceftazidine and clndamycin
D. Penicillin
E. Vancomycin
B. Cefotaxime and flucloxicillin
Need to cover for staph (fluclox) and is very sick. Especially because she has pleural effusion. Without pleural effusion penicillin would be correct. Cover strep pneumo and Haemophilus with cefotaxime.
A 10 year old girl with a background of vulvovaginitis presents with a 10 day history of dysuria and
vulvar pain associated with itching, predominantly at night. A swab grows Group A Streptococcus.
She is commenced on penicillin. What other treatment is most indicated?
A. Cephalexin
B. Mebendazole
C. Clotrimazole
D. Mupirocin
E. Trimethopri
B. Mebendazole
- Clotrimaxozole – for candida
- Treat for pinworm with pyrantol – combantrim
- Answer is mebendazole – suspect threadworm. Often worse at night!
A 10 year old boy presents following three separate early morning seizures, occurring within 30
minutes of waking. They commence as perioral numbness and progress to a generalized clonic tonic
seizure, lasting between 3 to 5 minutes. He is developmentally normal. Which of the following is
the most likely diagnosis?
A. Benign epilepsy with centrotemporal spikes
B. Benign occipital epilepsy
C. Childhood absence seizures
D. Juvenile myoclonic epilepsy
E. Familial generalized epilepsy
A. Benign epilepsy with centrotemporal spikes
- Onset is typically in mid-childhood (peak 7 years).
- Seizures are commonly nocturnal or early morning. In the centro-temporal (Rolandic) variety, they are usually focal motor or sensory phenomena related to the face, mouth or jaw. The occipital variety may have visual manifestations. Secondarily generalised tonic– clonic seizures may also occur.
- On EEG, spike discharges typically occur in the centrotemporal or occipital region.
- Treatment is only indicated in children with frequent or prolonged seizures. Low-dose carbamazepine or sodium valproate is used for 1–2 years given the spontaneous remission of seizures.
A 2 year old girl presents with tachycardia. Her heart rate is 210 bpm and stable and abruptly drops
to 140 bpm. An ECG shows a narrow complex tachycardia with visible P waves superimposed on T
waves. Which of the following is the most likely diagnosis?
A. Atrial ectopic
B. Junctional tachycardia
C. Sinus tachycardia
D. Wolff-Parkinson-White syndrome
E. Ventricular tachycardia
A. Atrial ectopic
A 16 year old boy presents with short stature and delayed puberty. His mother and father are 160
and 170cm tall respectively. A history of anosmia is elicited. Examination is normal aside from
nystagmous and abnormal mirror movements. Blood test results are as follows: LH 1 (<4), FSH 1
(<4), Testosterone 0.4 (0.4-3), GH 2 (<10), IGF 1 200 (50-330). Which of the following is the most
likely diagnosis?
A. Familial short stature
B. Delayed puberty and growth
C. Hypogonadotropic hypogonadism
D. Klinefelters
E. Isolated GH deficiency
C. Hypogonadotropic hypogonadism
Kallmann’s syndrome — Kallmann’s syndrome is characterized by hypogonadotropic hypogonadism and one or more nongonadal congenital abnormality, including anosmia, red-green color blindness, midline facial abnormalities such as cleft palate, urogenital tract abnormalities, synkinesis (mirror movements) and neurosensory hearing loss [4-6]. Hypogonadism in this syndrome is a result of deficient hypothalamic secretion of GnRH. The hypogonadism may be severe, mild, or even transient.
Most cases of Kallmann’s syndrome are sporadic [7], but familial occurrence also occurs. Inheritance is usually X-linked, as judged by the much greater number of cases in males than females. However, autosomal dominant [4] or recessive transmission can occur.
A 16 year old girl is referred from ophthalmology. She has decreased vision, flame haemorrhages
and a blood pressure of 240/140. An MRI of her abdomen demonstrates a phaeochromocytoma.
Which of the following is contraindicated?
A. Hydralazine
B. Labetolol
C. Phenoxybenzamine
D. Propranolol
E. Sodium nitroprusside
D) propanolol
- In phaeochromocytoma, alpha-1 activity is main instigator of HTN
- Propranolol would be bad to use as it is a beta blocker and can exaggerate the alpha activity and thus worsen hypertensive crisis.
- Labetalol good as has combined blockade (a, b)
- phenoxybenzamine = a-blocker
A 16 year old girl presents with rectal bleeding. A colonoscopy reveals over 200 polyps in her colon. A diagnosis of Familial Adenomatous Polyposis (FAP) is confirmed. What is her lifetime risk of colon carcinoma? A. 20% B. 40% C. 60% D. 80% E. 100%
E. 100%
Familial Adenomatous Polyposis Coli (FAP)
-AD defect in the APC gene (tumour suppressor gene)
- Adenocarcinoma of colon.
- 1/500 – 1/17000 (generally 1 in 8000)
- Polyps develop around 10yo (premalignant state)
- By definition >5 polyps, usually 100s-1000s.
Clinical:
Can be asymptomatic
PR bleeding, cramps.
Dx on colonoscopy, FamHx
If mutation known, can differentiate amongst family members.
Malignancy
Usually by age 40 (as young as 10yo)
100% will develop Ca
Surveillance – Scope every 6m until Surgery
Rx: prophylactic proctocolectomy
Which of the following may recur in a transplanted liver? A. Neonatal haemochromatosis B. Methylmalonic acidaemia C. Primary sclerosing cholangitis D. Wilson’s disease
C. Primary sclerosing cholangitis
PSC recurs in 20% post-transplant
Some journals now require pre-registration of trials for future publication. The reason for this is to: A. Independent analysis B. Increased peer review C. Decreased fraudulent reporting D. Decreased false results E. Decrease publication bias
E. Decrease publication bias
Drug companies are not publishing all the trial data that they submit to the FDA, and those trials that are published are more likely to show positive results.
Rising et al. compared all the New Drug Applications (NDAs) (the vehicle for initiating a new clinical trial) given to the FDA in 2001 and 2002 to subsequent published literature. They found that only about 3/4 of the trials were later published in journals, and those that were published were 5 times as likely to show favorable results for the drug being
tested by the drug company as those that were not:
A 3 year old boy with developmental delay, known butterfly vertebrae, severe gastroesophageal
reflux and feeding difficulties with a past history of tracheo-esophageal fistula and esophageal
atresia, presents with a sore elbow after someone pushed him into a door at kindergarten. His elbow
is generally tender with limited pronation. There is an X-ray with no fracture and all the ossification
centres in the humerus ossified except the lateral epicodyle (?curved radius). Which of the following
is the most likely diagnosis?
A. Fractured humeral epicondyle
B. Dislocated head of radius
C. Radial ulnar synostosis
D. Fractured ulna
C. Radial ulnar synostosis
VACTRL
Fifty percent of infants are nonsyndromic without other anomalies, and the rest have associated anomalies, most often associated with the VATER or VACTERL (vertebral, anorectal, [cardiac], tracheal, esophageal, renal, radial, [limb]) syndrome. These syndromes generally are associated with normal intelligence.
A 6 week old infant presents with jaundice and poor feeding. His bloods are as follows: bilirubin 170
(conj 70), ALT 200, ALP 600, glucose 1.2, cortisol 100, growth hormone 1.4 (low), thyroxine 9 (low),
insulin <2, liver ultrasound normal. Which of the following is the most likely diagnosis?
A. Panhypopituitarism
B. Glycogen storage disease
C. Biliary atresia
D. Alagille syndrome
E. Primary hypothyroidism
A. Panhypopituitarism
Newborn screening measures TSH and retests those with high TSH. The cut-off is 15 apparently
TSH based screening misses central causes of hypothyroidism, however this is rare and may present with other symptoms of panhypopit or midline defects.
A toddler presents unwell with fever. Findings include hepatomegaly, pancytopenia and a ferritin of
29500. Which of the following is the most likely diagnosis?
A. Neonatal haemochromatosis
B. Acute myeloid leukaemia
C. Haemophagocytic lymphohistiocytosis
D. Sideroblastic anaemia
E. Neonatal iron storage disesae
C. Haemophagocytic lymphohistiocytosis
Hemophagocytic Lymphohistiocytosis
• Familial (25%) and secondary forms – similar presentation - familial form usually children <4 years but secondary form can present at an older age
• Can be secondary to viruses (EBV, CMV, HHV-6, HHV-8, VZV, HSV, HIV, parvovirus, adenovirus); bacterial/fungal/spirochetal infections; malignancy; AI disorders (SLE, RA, PN); transplant recipients
• Presentation:
o Fever (90%), maculopapular/petechial rash (40%), weightloss, irritability
o Respiratory distress
o CNS involvement: aseptic meningitis (50%)
o Hepatosplenomegaly (80-90%), lymphadenopathy (40%)
o Severe immunodeficiency
• Cytokine dysfunction→ uncontrolled accumulation of activated T-lymphocytes and activated histiocytes (macrophages) in many organs
o Haemophagocytosis seen in bone marrow
• Diagnosis: fever + splenomegaly + lab findings
o Hyperlipidaemia, hypofibrinogenaemia, elevated transaminases, extremely elevated levels of CD25 (circulating IL-2 receptors released by activated lymphocytes), very high levels of serum ferritin (often >10000), cytopenias (esp pancytopenia; from hemophagocytosis in the BM), low/absent NK cells
• Treatment: treat underlying infection, supportive care. Rx: immunoochemotherapy, hematopoietic cell transplant best cure. Bad prognosis
• FHLH is ultimately fatal without allogenic stem cell transplant; in contrast, secondary HLH has excellent prognosis IF an infection is identified but poor prognosis if it is not found
In 22q11 deletion, which of the following is the most commonly associated cardiac anomaly? A. Tetralogy of Fallot B. Aortic stenosis C. Supravalvular aortic stenosis D. Pulmonary stenosis E. Interrupted aortic arch
A. Tetralogy of Fallot
The most common cardiac anomalies include: TOF (25%) interrupted aortic arch (15%) VSD, usually perimembranous malaligned VSD (15%) persistent truncus arteriosus (9%) isolated aortic arch anomalies (5%). Less common anomalies include pulmonary stenosis, ASD, AV canal defect, and transposition of the great arteries.
A 3 and a half year old child is involved in a car accident. He is restrained. He sustains a scalp
haematoma and abrasions to the chest wall, abdomen and pelvis. He is clinically diagnosed with a
compound fracture of the left femur. His vital signs are as follows: Sa02 100%, RR 40, HR 88, BP
127/70, cap refill <2 secs, GCS intact.
Which of the following is most consistent with his vital signs?
A. Fractured femur
B. Liver laceration
C. Pelvic fracture
D. Intracranial injury
E. Abdominal trauma
??E. Abdominal trauma
?head injury (hypertension, bnut otherwise normal obs)
Hypovolemia is the most common cause of shock in the pediatric trauma patient, and its early recognition and treatment are critical during trauma resuscitation. Compensated shock occurs when there has been significant blood loss, but the blood pressure has been maintained by tachycardia and vasoconstriction. Uncompensated shock manifests with hypotension in addition to tachycardia.
Tachycardia is usually the first sign of hypovolemia in the child. Due to the physiologic reserve in children, blood pressure may be maintained despite a loss of up to 45 percent of the circulating blood volume. Therefore, the trauma patient who is cool and tachycardic should be considered to be in shock until proven otherwise.
A 6 week old infant has a haemangioma on the left thigh (pictured) noted at 1 week of age. What is
the chance of complete resolution by 5 years of age?
A. 20%
B. 40%
C. 60%
D. 80%
E. 100%
D. 80%
Which of the following best discriminates childhood bipolar disorder from ADHD? A. Euphoria B. Mood swings C. Irritability D. Aggression
A. Euphoria
ADHD core symptoms of - Inattention, hyperactivity and impulsivity
- present > 6 months
- present more than one setting (e.g school and home)
- result in significant impairment in daily living
- some symptoms must begin < 7 years of age (diagnosis difficult < 4 years)
Subtypes: predominantly inattention, predominantly hyperactive-impulsive
BIPOLAR DISORDER
- Alternating mania and depression (can be frequent cycling)
- Often begins in adolescence (family history important)
- Features of mania/elevated mood:
o Inflated self esteem
o Decreased need for sleep
o Increased talk/pressure speech
o Flight of ideas, racing thoughts
o Distractable
o Goal directed acitivity – social, work, sexual
o High risk pleasurable activities
A term neonate is in NICU with hypotonia, weak respiratory effort, and arreflexia. No facial weakness. CXR – shows massive cardiomegaly.
What is the most likely diagnosis?
A) Congential myotonic dystrophy B) Nemaline core myopathy C) Pompe’s disease D) Spinal muscular atrophy E) Mitochondrial myopathy
C) Pompe’s disease
- glycogen storage disease (build of glycogen in lysosymes).
- Hypotonia, weakness and areflexia.
- Cardiomyopathy (huge heart).
- Big liver and tongue too.
- CK↑.
- Myopathic EMG.
- Treatment is enzyme replacement.
An 8 week old child presents to the emergency department. She has a history of an idiopathic
seizure disorder and is treated with phenobarbitone. She has presented because she has had a run
of 4 seizures in the past 24 hours. When you review the child she looks well. Her phenobarbitone
level is 24 (40-80).
Your next step is to:
A. Load with phenytoin
B. Load with phenobarbitone
C. Increase the daily dose of phenobarbitone
D. Change to Vigabatrin
B. Load with phenobarbitone
As per AMH, steady-state plasma concentration may not be achieved for 2–4 weeks, maybe even longer in neonates. This patient is symptomatic – needs more drug in system quickly.
A 6 year old girl receiving chemotherapy for acute lymphoblastic leukaemia presents with fever, bruising, petechiae, and oral mucosal bleeding. Her platelet count is 4 x 10^9 [150-400] and her blood group is A-. A platelet transfusion is indicated. However, the transfusion service does not have irradiated A- platelets
available.
A. Irradiated Group A, Rhesus positive platelets from single donor
B. Irradiated Group A, Rhesus positive platelets from pooled donors
C. Irradiated Group O, Rhesus negative platelets from single donor
D. Irradiated Group O, Rhesus negative platelets from pooled donors
E. Un-irradiated Group A, Rhesus negative platelets from single donor
C. Irradiated Group O, Rhesus negative platelets from single donor
Anthean Greenway
• Must be rh negative – most important. Risk of antiD
• Irradiation due to risk of GVHD risk
• Platlets used to be in plasma but now a different medium and not in plasma
• Thinks answer C
• Should be single donor – important because of HLA sensitisations
Email from Gemma Creighton the haem reg:
I would avoid any Rhesus positive platelets as, she is Rhesus negative and risk of Rhesus immunisation by giving RH positive products. I’d definitely not want to do that.
In practice I would avoid the O platelets, as they contain small amounts of anti-A and anti-B, but sometimes you can’t. Often Red Cross titres the amount of anti-A and anti-B and can potentially give low titre product. A single donor is less risk of exposure to anti- A than a pooled donor unit.
Need to give irradiated products as receiving chemo and nucleoside analogues - see irradiation info - if interested.
So would give (C) - hope that agrees with what the answer is.
A baby transported via fixed wing aircraft. The child is stable with normal oxygen saturations prior
to departure. On ascension, the child’s oxygen saturations fall. The baby still looks comfortable on
the ventilator. Which of the following is the most appropriate next action?
A. Increase the FiO2
B. Increase the inspiratory time
C. Increase the PEEP
D. Increase the PIP
E. Do nothing
A. Increase the FiO2
- higher PIP mainly drop CO2, decondarily increase O2 (due to increased lung recruitment)
- increase I time = increase oxygenation may also increase CO2
Transport Physiology
On fixed- or certain rotary-wing transports, the patient may suffer adverse physiologic effects from altitude. With increasing altitude, the barometric (atmospheric) pressure decreases and gases expand. As the barometric pressure drops and gas expands, the partial pressures of ambient oxygen (PO2) and, consequently, arterial oxygen (PAO2) decrease. For example, at 8,000 feet—an elevation at which unpressurized airplanes may fly, as well as the effective cabin altitude for many pressurized airplanes flying at 35,000 to 40,000 feet—the barometric pressure, PO2, PAO2, and arterial oxygen saturation fall to 565 mm Hg, 118 mm Hg, 61 mm Hg, and 93%, respectively. (In comparison, the barometric pressure, PO2, PAO2, and arterial oxygen saturation are 760 mm Hg, 159 mm Hg, 95 mm Hg, and 100% at sea level.) Although healthy individuals usually tolerate these changes well, patients with respiratory insufficiency, significant blood loss, or shock may decompensate and should receive supplemental oxygen.
Gases expand 10-15% at the few thousand feet at which helicopters typically fly, and approximately 30% at 8,000 feet. Gases within the body itself also expand as the altitude increases. Gas expansion must be appreciated during transport via air of a patient with a pneumocephalus, pneumothorax, bowel obstruction, or another condition involving entrapped gas. Prior to transport, a pneumothorax should be decompressed, and a nasogastric tube inserted for ileus.
A child with known congenital adrenal hyperplasia is currently managed on hydrocortisone and
fludrocortisone. Her weight and height are on the 50th centile, and her blood pressure is slightly
elevated. Her 17-hydroxyprogesterone is 4 (<4). Which of the following is the most appropriate
management?
A. Decrease fludrocortisone
B. Decrease hydrocortisone
C. Increase fludrocortisone
D. Increase hydrocortisone
E. Start an antihypertensive
D. Increase hydrocortisone
Treatment of congenital adrenal hyperplasia with hydrocortisone and fludrocortisones
Defective conversion of 17-hydroxyprogesterone to 11-deoxycortisol accounts for more than 95 percent of cases of congenital adrenal hyperplasia [1]. This conversion is mediated by 21-hydroxylase.
Therapy of CYP21A2 deficiency is directed toward providing glucocorticoid in sufficient doses to reduce the excessive corticotropin-releasing hormone (CRH) and corticotropin (ACTH) secretion and hyperandrogenemia, so that growth, sexual maturation, and, later, reproductive function, are normal.
In patients with the salt-losing form of the disorder, mineralocorticoid is given to restore serum electrolyte concentrations, and extracellular fluid volume to normal. These goals can be difficult to achieve without overtreatment, and its attendant risk of growth retardation and other clinical manifestations of Cushing’s syndrome
Glucocorticoid replacement is necessary in children who have classic CYP21A2 deficiency and in symptomatic patients with nonclassic CYP21A2 deficiency. The goal of therapy is to replace deficient steroids while minimizing adrenal sex hormone and glucocorticoid excess. Glucocorticoid is usually administered as hydrocortisone (cortisol) in a dose of 12 to 18 mg/m2 body surface area per day. Doses are greater than normal cortisol levels. For older adolescents and adults, long-acting glucocorticoids such as dexamethasone or prednisone are the preferred treatment. When given at bedtime, these drugs effectively suppresses ACTH secretion for much of the next day. However, the longer duration of action and greater potency of dexamethasone may increase the risk of overtreatment,
Mineralocorticoid replacement is necessary in patients who have the classic form of CAH, whether or not it is the salt-losing form. Mineralocorticoid is given as fludrocortisone, in a dose sufficient to restore normal serum sodium and potassium concentrations. Excessive dosing can induce hypertension, hypokalemia, and possibly, impaired growth, while underdosing also can lead to poor growth with failure-to-thrive.
In addition, inadequate mineralocorticoid replacement may lead to increased adrenal androgen production. This is because patients may be chronically volume depleted, which is clinically inapparent but results in persistent overproduction of renin and angiotensin II. Angiotensin II can stimulate early steps in the steroidogenic pathway, leading to higher adrenal androgen synthesis. For all of these reasons, mineralocorticoid therapy may benefit patients with either the classic salt-losing form or the classic non-salt-losing (simple virilizing) form of CYP21A2 deficiency.
The usual pediatric dose of fludrocortisone is 0.05 to 0.20 mg per day. Infants with the salt-losing form of CYP21A2 deficiency may require higher doses of fludrocortisone (occasionally up to 0.30 mg per day) and also require sodium chloride supplementation of 1 to 3 g per day (about 17 to 51 mEq per day).
Response to therapy is monitored by measuring serum 17-hydroxyprogesterone, androstenedione, plasma renin activity or direct renin, as well as growth velocity and the rate of skeletal maturation. Blood samples for monitoring therapy should be obtained at a consistent time in relation to glucocorticoid dosing, optimally in the morning to reflect peak concentrations.
A serum 17-hydroxyprogesterone concentration of 400 to 1200 ng/dL (12-36 nmol/L) with serum androstenedione concentrations appropriate for the patient’s age and sex, measured in the morning before the glucocorticoid is taken, is a reasonable target. The serum tests serve as markers of the adequacy of treatment and of patient adherence to it, and along with the growth record provide the basis for adjustments in the doses of hydrocortisone. Simply adjusting the glucocorticoid dose to normalize the serum 17-hydroxyprogesterone value is insufficient because of the risk of iatrogenic Cushing syndrome and since androgen excess may persist because of avid adrenal conversion of 17-hydroxyprogesterone to androgens. Plasma renin activity should be in the normal range for age. In patients with salt-losing and continued androgen excess, fludrocortisone and salt tablets dose should be adjusted to normalize renin before increasing the glucocorticoid dose.
A 3 week-old infant presents with several hour history of poor feeding and is floppy. On examination he is hypotonic with absent reflexes.
What is the most likely cause for this presentation?
A) Botulism B) Guillain-Barre syndrome C) Spinal muscular atrophy D) Congenital myotonic dystrophy E) Myasthenia gravis
A) Botulism
toxin mech: inhibition of acetylcholine release into synaptic junction
Acute, flaccid paralysis caused by the neurotoxin produced by Clostridium botulinum - Suspect in children 2–9 months of age with constipation and rapid onset of weakness, particularly with ophthalmoplegia and The toxin blocks neuromuscular transmission and causes death through airway and respiratory muscle paralysis. Botulinum toxin is carried by the bloodstream to peripheral cholinergic synapses, where it binds irreversibly, blocking acetylcholine release and causing impaired neuromuscular and autonomic transmission./respiratory weakness.
The most striking epidemiologic feature of infant botulism is its age distribution: in 95% of cases the infants are between 3 wk and 6 mo of age, with a broad peak from 2 to 4 mo of age. • Uncommon and often unrecognised. Identified risk factors for the illness include breast-feeding, the ingestion of honey, and a slow intestinal transit time (<1 stool/day)
- Botulinum toxin is distributed hematogenously. Because relative blood flow and density of innervation are greatest in the bulbar musculature, all forms of botulism manifest neurologically as a symmetric, descending, flaccid paralysis beginning with the cranial nerve musculature. It is not possible to have botulism without having multiple bulbar palsies: poor feeding, weak suck, feeble cry, drooling, and even obstructive apnea
- In infants, usually, the 1st indication of illness is a decreased frequency or even absence of defecation, although this sign is frequently overlooked. Parents typically notice inability to feed, lethargy, weak cry, and diminished spontaneous movement. Dysphagia may be evident as secretions drooling from the mouth. Gag, suck, and corneal reflexes diminish as the paralysis advances. Oculomotor palsies may be evident only with sustained observation. Paradoxically, the pupillary light reflex may be unaffected until the child is severely paralyzed, or it may be initially sluggish. Loss of head control is typically a prominent sign. Respiratory arrest may occur suddenly from airway occlusion by unswallowed secretions or from obstructive flaccid pharyngeal musculature
A boy with a history of biliary atresia, presents with splenomegaly and a platelet count of 60.
Gastroscopy reveals oesophageal varices [told this but picture also provided].
In Australia, which of the following is the first line treatment for primary prevention of bleeding from
oesophageal varices?
A. Defibrotide
B. Octreotide
C. Nitrates
D. Propranolol
E. Vasopressin
B) octreotide
For prophylaxis:
o Vasopressin (or analogue, e.g. DDAVP) has been commonly used, and is though to act by increasing splanchnic vascular tone and thus decreasing portal blood flow. (Vasopressin’s use may be limited by side effects of vasoconstriction, such as impaired cardiac function and perfusion to the heart, bowel and kidneys.)
o GTN (skin patch) has also been used to decrease portal pressure.
o The somatostatin analogue octreotide decreases splanchnic blood flow with fewer side effects.
Propranolol for long term treatment in adults (limited evident in children)
ECG given [axis between +90 and 180 degrees, tall Ps, biphasic Ts in V1 to ~V3, I don’t think there was
any voltage criteria for ventricular hypertrophy and Ts were negative in V1]
In which condition are you most likely to find these changes? [no clinical scenario or age given]
A. AVSD
B. Ebstein’s anomoly
C. Eisenmeger’s
D. TAPVR
E. Tetralogy of Fallot
?A. AVSD
A 15 year old girl with anorexia nervosa has a body mass index (BMI) of 15. She denies an eating
disorder. Which of the following is likely to be the most effective treatment?
A. Antipsychotic
B. CBT
C. Interpersonal therapy
D. Maudsley method of family therapy
E. SSRI
D. Maudsley method of family therapy
- RCH tends to talk about Family Therapy (Maudsley Method) and not favour medications as heavily
- Generally speaking, CBT and medications show more benefit in bulimia and binge eating and have been disappointing in anorexia
Maudsley Method - Family Therapy (but involves aspect of CBT and Parents take control of refeeding their child in a structured manner
• Priority given to providing adequate calories
• Goal-structures and behavioural expectations such as eating 100% of food and achieving a rate of weight gain with contingencieas if goals not achieved.
• Promising results in adolescents
• There is a transition from parental control to adolescent taking control
An 18 month old boy presents with recurrent infections. Which of the following is most likely to be found in X-linked agammaglobulinaemia? A. Empty tonsillar bed B. Splenomegaly C. Lymphadenopathy D. Candidiasis E. Diarrhoea
A. Empty tonsillar bed
A three-year-old boy presents with a four-week history of irritability and fever. He was previously
well and is appropriately immunised. He has a five-year-old sister who is well. There is no history of
overseas travel.
At the start of the illness he was diagnosed with pharyngitis and prescribed amoxycillin. After taking
the amoxycillin for five days, the fevers persisted and a truncal rash appeared. The amoxycillin was
stopped and the rash resolved, but has reappeared intermittently, especially when he is highly
febrile (see photograph shown below).
Examination shows a miserable, irritable boy who is febrile (38.4°C), tachycardic (140/minute), with
bilateral cervical lymphadenopathy and dry cracked lips. His spleen is tippable. He has a 2/6 ejection
systolic murmur at the apex which does not radiate. The remainder of the physical examination is
normal.
Investigations show:
- Haemoglobin 85 g/L [110-140]; white cell count 24.8 x 10^9/L [4.0-11.0]; neutrophils 18.2 x 10^9/L [1.0-4.0]; platelet count 720 x 10^9/L [150-500]
- Erythrocyte sedimentation rate (ESR) 110 mm/hr [<20]
- IgG 14.1 g/L [4.2-11.9]; IgA 2.3 g/L [0.2-1.6]; IgM 2.1 g/L [0.4-1.9]
Which one of the following is the most likely diagnosis?
A. Epstein-Barr virus infection.
B. Kawasaki disease.
C. Mycoplasma pneumoniae infection.
D. Rheumatic fever.
E. Systemic onset juvenile chronic arthritis.
E. Systemic onset juvenile chronic arthritis.
A well father has brought his two boys to see you. Both boys have Myotonic Dystrophy. No genetic
testing has been performed. The boy’s mother is asymptomatic. Which of the following is the most
likely explanation?
A. Non paternity
B. Non penetrance
C. Gonadal mosaicism
D. Anticipation
D. Anticipation
The mother is nearly always affected in congenital myotonic dystrophy but previously diagnosed in only half of all cases
Myotonic dystrophy: premutation carriers (50-150 rpts) have cataracts and mild myotonia
- Myotonic dystrophy – CTG,CTG,CTG
- Progressive wasting of distal muscles (esp hand intrinsic muscles and thenar/hypothenar eminences) – ie EXCEPTION to prox>distal rule for most myopathies. Prox mm involved later as disease progresses.
- Also assc with cataracts, early male baldness, low IgG, intellectual impairment, endocrinopathies (thyroid, addisons, DM, early/late puberty, testicular atrophy/T deficiency, male infertility)
- Arrhythmias but not cardiomyopathy.
Myotonic dysptrophy
• AD (triplet repeat)
• hypotonic from birth (↓fetal movements, polyhydramnios), ± arthrogryphosis
• facial weakness, difficulty feedings and respiratory difficulties
• distal > proximal weakness (unusual for a myopathy)
• myotonia about 5yo
• reflexes OK (cf SMA)
• cataracts
• 50% have ↓ IQ
• Endocrine abnormalities (low thyroid, adrenals, pancreas, testosterone)
• Cardiac conduction defects (don’t tend to develop cardiomyopathy unlike other myopathies)
• Neonatal form is more severe with generalised weakness including respiratory
A 6 year old boy presents for review. He sleep walks most nights at 10:45pm. Which of the
following is the most appropriate recommendation to the boy’s parents?
A. Scheduled waking at 10:30pm
B. Behavioural management
C. Make him go to bed earlier
D. Diazepam
E. Reassurance
B. Behavioural management
A 4 month-old boy presents with increased work of breathing and he is noted to have decreased breath sounds on the R side of his chest.
A CXR is shownm[Hyperlucent and ?hyperinflated R side with ?mediastinal shift, trachea in midline]
What is the most likely diagnosis?
A) Bronchogenic cyst B) Pulmonary hypoplasia C) Vascular ring D) Congenital lobar emphysema E) Inhaled foreign body
D) Congenital lobar emphysema
Location -LUL -RML/RUL -<10% lower lobes (hyperlucent, hyperexpanded)
Clinical
- present in the 1st 2 weeks of life
- tachypnoea
Treatment
- symptomatic: resect
- asymptomatic: conservative
Notes:
- dilatation of 1 lobe
- 50% cause unknown
- 25% obstruction (ball-valve mechanism)
A 5-year-old boy comes to see you with gradual worsening of retro-sternal pain and increasing difficulty swallowing. He can no longer swallow solids, and has a liquid diet. Apart from a history of mild eczema, there is no other history of note. He has previously been on omeprazole with no
improvement of his symptoms. What is the most likely diagnosis?
A. Achalasia
B. Sliding hiatal hernia
C. Coeliac disease
D. Gastritis
E. Eosinophilic oesophagitis
E. Eosinophilic oesophagitis
Eosinophilic oesophagitis
• Most common in Caucasian males
• Hx of atopy in 50%, pos atopic fhx in 75%
• Sx: feeding disorders (median age 2.0), vomiting (median age 8.1), abdominal pain
• (median age 12.0), dysphagia (median age 13.4), and food impaction (median age 16.8)
• Ix: serum eos usually normal, IgE can be slightly high
• characteristic clinical features and large numbers of eosinophils in the esophagus on pathologic examination (≥15 eosinophils per high powered [400x] field in at least one specimen) despite acid suppression with a PPI for one to two months. The criteria also include normal gastric and duodenal mucosal biopsies and the exclusion of other causes.
• Rx: cortisone, PPI, esophageal dilatation, elimination diet (? assoc with food allergy)
• Prognosis: probably persists into adulthood, 10% developed tolerance to food allergies
Achalasia:
• 1:100000 (usually 25-60y)
• Disease of unknown cause in which there is a loss of peristalsis in the distal esophagus and a failure of lower esophageal sphincter (LES) relaxation.
• Symptoms of achalasia are due primarily to the defect in LES relaxation → functional obstruction of the esophagus that persists until the hydrostatic pressure of the retained material exceeds the pressure generated by the sphincter muscle
• Insidious onset, gradual progression of sx.
• Dysphagia for liquids most strongly suggests diagnosis!
• Dx: barium swallow (dilated esophagus that terminates in a beak-like narrowing), fluoroscopy, manometry
o (3 characteristic manometric features of achalasia: elevated resting lower esophageal sphincter (LES) pressure (above 45 mmHg); incomplete LES relaxation after a swallow (S); aperistalsis in the smooth muscle portion of the body of the esophagus. Swallows may elicit no esophageal contraction or may be followed by simultaneous contractions. The esophagus may also contract spontaneously in a simultaneous fashion. In some cases, the simultaneous esophageal contractions have amplitudes >60 mmHg, a condition known as “vigorous” achalasia.)
• Rx: Medical (nitrates, Ca channel blockers, Botox), Dilatation of LES, myotomy
A healthy and otherwise well child presents with a history of three proven meningococcal infections.
Which of the following investigations is most likely to lead to a diagnosis?
A. B and T cell subtypes
B. Neutrophil function
C. Properedin
D. CH50 & CH100
D. CH50 & CH100
The graph below represents the exposure to particulate matter and the corresponding relative risk
of cardiovascular disease.
See Paper 2 2019: Q73.
“Level of Exposure to Fine Particulate Matter and the Risk of Death from Cardiovascular Causes in
Women”
Which of the following is the best explanation for the variation in range of relative risk as the
amount of exposure to particulate matter increased?
A. Variation between rural and urban exposure
B. Small numbers of people exposed to high levels of particulate matter
C. Increased causal association
B. Small numbers of people exposed to high levels of particulate matter
The confidence limits are widest at highest levels because there are very few women in the study who have had this level of exposure. Confidence limits narrow with increased number of observations or decreased variance
Which of the following is the most common side effect of cyclosporine?
A. Hirsutism
B. Hypertension
C. Renal impairment
A. Hirsutism
A child presents with a history of recurrent renal stones. Which of the following is the most
appropriate dietary management for prevention of stones?
A. Reduce dietary sodium
B. Reduce dietary oxalate
C. Increase dietary oxalate
D. Reduce dietary urate
E. Reduce dietary calcium
A. Reduce dietary sodium
Dietary measures to reduce urinary calcium excretion include the following:
- Low-sodium diet enhances sodium and calcium renal tubular reabsorption, thereby reducing urinary calcium excretion.
- Dietary calcium is not restricted
- Vitamin D supplementation is avoided because elevated serum calcitriol (1,25-dihydroxyvitamin D3) levels can enhance urinary calcium excretion
Thiazide diuretics should be considered in a child or adolescent with recurrent calcium stones if dietary measures over a three to six month period fail to reduce urinary calcium levels. Thiazide diuretics (ie, chlorothiazide or hydrochorothiazide) enhance sodium and calcium reabsorption in the distal renal tubule leading to a reduction in urinary calcium excretion
A 5 year old boy presents with boils on his arm and chest. He is systemically well. Which of the
following is the most appropriate treatment?
A. Clindamycin
B. Cephalexin
C. Muipirocin
D. Vancomycin
E. Flucloxicillin
?C. Muipirocin
A 10 year old girl presents following multiple episodes of syncope. Her ECG is shown below.
See Paper 2 2019: Q77. - long qt
Which of the following is the most appropriate treatment? A. Amiodarone B. Flecainide C. Ablation D. Propranolol E. Defibrillation
D) propanolol
Torsade de Pointes almost pathognomic for long QT
Congenital Long QT syndromes
• Congenital LQTS due to ion pump mutations, most commonly (1,2,4 6) affect the outflow of K, so can’t re-polarise efficiently long QT
o Long QT 3 is excessive sodium inward current
• 25% are sporadic, otherwise mostly AD, some AR
• History difficult to distinguish between seizure and cardiac syncope
o Clues: syncope or seizure after upset, exertion of exposure to water / swimming
o ECG on first presentation of seizure
• Mortality is high if untreated (20% in year after first episode, 50% in 10 years)
• The actual QT interval is not predictive of risk of death
• Ventricular bigeminy in known long QT = impending torsades!
• Risk Factors: Deaf, Sx in infancy, history of cardiac arrest, long QT on ECG at rest
o ECG at rest may miss prolonged QT (in 5-10%) so need to do exercise ECG
Management
• Risk stratification possible on basis of QTc, sex, age and genotype
• Beta blockers: ↓PR response to exercise. Are mainstay of therapy, most patients will be asymptomatic and Sudden Cardiac Death rate drops dramatically (to 4%). Titrate to maximum tolerated dose
• PM if become brady on b-blockers
• ICD if not managed on b-blockers (repeated syncope) or cardiac arrest.
A woman has been newly diagnosed with TB with a positive sputum sample. Her 3 month old son
has a normal CXR. He has not had a BCG and his Mantoux is negative. Which of the following is the
most appropriate management strategy for the child?
A. Prophylaxis isoniazid for 3 months and then a repeat mantoux
B. Observation
C. Early morning gastric aspirates
D. Bronchoalveolar lavage
E. Repeat CXR and mantoux in 3 months
A. Prophylaxis isoniazid for 3 months and then a repeat mantoux
- High risk exposure
- If knew at birth would do the same thing
- If mantoux positive at 3 months – give total 6 months isonazid and rpt cxr. Continue to monitor if baby well
- SE: isoniazid = peripheral neuropathy. Give B6 to reduce SE. (don’t usually give B6 to kids as often have adequate stores but in baby probably would)
- Don’t vaccinate as want to be able to interpret mantoux
- Baby this age would not be symptomatic even if they had respiratory TB. Need to monitor carefully clinically and with CXR
- TB can be congenital infection too
A 15 year old girl with a known diagnosis of Turner’s syndrome and a mosaic on karyotype (45 X, 46
XX) presents for pubertal induction for delayed puberty. Which one of the following investigations is
essential to perform prior to commencing treatment?
A. Bone age
B. FSH
C. Oestradiol level
D. IGF-1
E. Pelvic Ultrasound
? E. Pelvic Ultrasound
A 12 month old, 10 kg child with gastroenteritis is judged to be 8% dehydrated. Which of the
following is the minimum fluid intake the child should receive in the next 24 hours?
A. 800mL
B. 1000mL
C. 1800mL
D. 2000mL
E. 2500mL
C. 1800mL
Maintenance: 10kg child @ 100ml/kg/day = 1000ml
Deficit: 8% x 10kg = 800ml
Hence need to replace 1800ml over 24 hours – answer D
An immunized child presents with bacterial tracheitis. Which of the following is the most likely causative organism? A. Streptococcus pneumoniae B. Staphylococcus aureus C. H. influenzae Type B D. Non-typeable H. influenzae E. Moraxella catarrhalis
B, staph aureus
- Staph causes up to 50% - most common
- Next: Strep pneumonia
- Next: Haemophilus influenzae
- Next: Group A strep
A child undergoes a water deprivation test for investigation of suspected diabetes insipidus. Her
results at the onset of the test are as follows: serum Na 150; serum osmolality 316; urine osmolality
260. Which of the following is the most appropriate next step?
A. Stop the test
B. Diagnose central diabetes insipidus
C. Administer IV saline
D. Give water to correct hypernatraemia and reschedule the test for a day when the patient is
well hydrated
E. Administer DDAVP
E. Administer DDAVP
Already meets criteria for DI. Need to assess whether central/peripheral
Diabetes insipidus is present when the serum osmolality is raised (>295milliOsmol/kg) with inappropriately dilute urine (urine osmolality < 700milliOsmol/kg). The serum sodium is often elevated due to excess free water losses.
