Pediatrics Best R.

1) The following laboratory results were returned in a 6 week old boy admitted with 6 days of severe projectile vomiting: pH 7.51 (7.35-7.45) PO2 12 KPa (95 mmHg) PCO2 4.7 KPa (35 mmHg) Blood Urea 11 mmol/l Sodium 131 mmol/l Potassium 3 mmol/l Chloride 83 mmol/l Which of the following is true concerning this patient? Available marks are shown in brackets 1 ) He has respiratory alkalosis 2 ) He is likely to have a bulging anterior fontanelle 3 ) He should be resuscitated immediately with normal saline He should be commenced immediately on half strength soy protein, low lactose 4) formula 5 ) X-ray of abdomen is likely to show dilated loops of small bowel

[0] [0] [100] [0] [0]

Comments: He has a metabolic, rather than respiratory alkalosis as CO2 is not reduced. He is likely to have a sunken fontanelle as he is very dehydrated.
Pyloric stenosis would highly likely as because of the age and hypochloraemic, hypokalaemic metabolic alkalosis. He should be resuscitated with normal saline first. Dilated bowel would not be expected, as pyloric rather than small bowel obstruction is present. 2) A 7 year old girl presents with a 3 day history of rash and ankle swelling. She had a cold 4 weeks previously, but has otherwise been healthy. 39+1/40, 2.96kg. No neonatal problems. No drugs or medications. Fully immunized. On examination she has palpable non-blanching purple spots 1-4 mm in diameter especially over the shins and buttocks. Her left ankle is swollen, warm and tender, with restricted movement. What is the most likely diagnosis? Available marks are shown in brackets [0] 1) Viral infection 2) Trauma/ child abuse [0] 3) Streptococcal infection [0] 4) Vasculitis eg HSP [100] 5) Thrombocytopaenia [0] Comments: The history is of preceding URTI followed by vasculitis on the shins and buttocks, and ankle swelling. This is classical of Henoch-Schonlein purpura. The pathological lesion is a vasculitis, hence the lesions are often palpable. In contrast thrombocytopaenic purpura are not raised. The classical features are rash, joint swelling, haematuria, and GI symptoms (vomiting, abdominal pain, PR bleeding, occasional intussusception).

Pediatrics Best R.

3) A 2 month old baby is admitted with seizures and failure to thrive. Follwoing a seizure he is found to have a blood glucose concentration of 1.2 mmol/l. Other biochemistry reveals a lactic acidosis and hyperlipidaemia. Examination reveals hepatomegaly with palpable kidneys. What is the most likely diagnosis? Available marks are shown in brackets 1) Gaucher's disease 2) McArdle's disease 3) Niemann-Pick disease 4) Pompe Disease 5) Von Gierke disease

[0] [0] [0] [0] [100]

Comments: This baby has hypoglycaemia accounting for fits, associated with lactic acidosis and hepatomegaly which would point to a diagnosis of von Gierke's disease. This is a glycogen storage disease due to deficiency of glucose-6-phosphatase. Glycogen is stored in both liver and kidney causing enlargement. Both Gaucher and Niemann-Pick disease are lipid storage disorders. McArdle's disease is a glycogen storage disease affecting muscle rather than liver as is Pompe's disease. Neither would be expected to produce profound hypoglycaemia.

4) A 15 month old child with flexural eczema presents with a 3 day history of cough and wheeze. There is a family history of hay fever. On examination his saturations are 92% in air, with a respiratory rate of 55/min and moderate recession. The heart rate is 150/min. What is the most likely diagnosis? Available marks are shown in brackets 1) Gastroesophageal reflux 2) Allergic rhinitis 3) Sinusitis 4) Asthma 5) Croup

[0] [0] [0] [100] [0]

Comments: This child has virus-induced wheeze on an atopic background. He may well be an early asthmatic, in which case recurrent episodes of cough and wheeze can be anticipated.

Pediatrics Best R.

5) An overweight twelve year old boy attends A+E complaining of hip and knee pain after a minor fall from his bike. On examination his knee appears normal but there is restricted range of movement at the hip. The likely diagnosis is: Available marks are shown in brackets 1) Chondromalaichae patella 2) Osteosarcoma 3) Perthes disease 4) Septic arthritis 5) Slipped upper femoral epyphesis

[0] [0] [0] [0] [100]

Comments: Slipped upper femoral epyphesis is most common in obese, adolecent boys with a positive family history. 25% are bilateral. Presentation may be with a coxalgic externally rotated gait, decreased internal rotation, thigh atrophy and hip, thigh and knee pain.
6) A 15 year old boy presents with a painful lower right arm after falling from his motorbike. X-ray reveals a fracture of the distal radius. What is the name for this fracture? Available marks are shown in brackets 1) Bennett's fracture 2) Colle's fracture 3) Galeazzi' s fracture 4) Monteggia' s fracture 5) Pott's fracture

[0] [100] [0] [0] [0]

Comments: Bennett's fracture is an intra-articular fracture of the base of the first metacarpal. Galeazzi's fracture involves the radial shaft with dislocation of the distal radioulnar joint. Pott's fracture is a general term applied to fractures around the ankle.

Pediatrics Best R.

7) A 12 day old girl presents with poor feeding, vomiting and lethargy. Born at 37+6/40, 2.98kg, no neonatal problems. Uncomplicated pregnancy and delivery. No drugs or medications. No immunizations. No FH/SH of note. On examination she is thin and wasted. She has occasional twitching of the eyelids and mouth. She responds slightly to voice. Weight <3%, OFC 25%, temperature is 36.5C (tympanic), RR 40/min, HR 150/min. Chest clear, no murmur. No organomegaly. No rash or dysmorphic features. What is the most likely diagnosis? Available marks are shown in brackets 1) Congenital adrenal hyperplasia 2) Congenital heart disease 3) Inborn errors of metabolism 4) Sepsis

[0] [0] [100] [0]

Comments: This child is dehydrated, vomiting and lethargic. Examination suggests possible subtle fits. Although infection and a cardiac abnormality are possible, a metabolic condition should be strongly suspected. Evaluation should include electrolytes, NH4, blood gases, serum and urine aminoacids, and urine organic acids.
8) 7 year old girl presents with high fever and severe left-sided throat pain. She has had difficulty in swallowing over the last 2 days, and has been finding it increasingly uncomfortable to open her mouth. Her voice is muffled and she dribbles saliva. She was born at 41/40 gestation weighing 4.0kg and there were no neonatal problems. On examination she looks ill. Her temperature is 40.2C (tympanic), RR 15/min and HR 100/min. ENT examination shows left tonsillar enlargement and exudate, with a uvula deviated to the right. What is the most likely diagnosis? Available marks are shown in brackets 1) Retropharyngeal abscess 2) Foreign body aspiration 3) Anaphylaxis 4) Croup 5) Peritonsillar abscess

[0] [0] [0] [0] [100]

Comments: The history suggests a peritonsillar abscess (quinsy) on the left side, a complication of Group A Strep. sore throat. A fever greater than 39.4C is associated with severe disease, and treatment is by surgical drainage.

Pediatrics Best R.

9) Feeding difficulties in the neonatal period may be due to all of the following except: Available marks are shown in brackets 1) Prematurity. 2) Hiatus hernia. 3) Cerebral birth trauma. 4) Physiological jaundice 5) Congenital heart disease

[0] [0] [0] [100] [0]

Comments: All of the options are associated with feeding difficulty except for physiological jaundice which is benign, shortlived and not generally associated with symptoms. 10) Features of Downs syndrome include all of the following except: Available marks are shown in brackets 1) A third fontanelle. 2) Duodenal atresia. 3) Ataxic gait. 4) Increased incidence of hypothyroidism. 5) Susceptibility to acute leukaemia

[0] [0] [100] [0] [0]

Comments: Cerebellar dysfunction is not a feature of Downs syndrome, however an Alzheimers like syndrome of memory loss and cognitive decline develops when patients reach the mid thirties. 11) Which of the following statements regarding messenger RNA (mRNA) is correct? Available marks are shown in brackets 1 ) mRNA never contains introns. 2 ) mRNA is translated into proteins in the nucleus. 3 ) mRNA contains the bases cytosine and thymine. Reverse transcriptase uses mRNA as a template to produce complementary 4) DNA. 5 ) mRNA is used in the Southern blotting technique.

[0] [0] [0] [100] [0]

Comments: The structure of mRNA is similar to DNA except that uracil replaces thymine as one of the bases. Both coding (exons) and non-coding regions of DNA are initially transcribed into mRNA. Splicing is required for mature mRNA to be produced only consisting of introns. Translation occurs in the cytoplasm. Southern blotting is a technique that uses denatured fragments of DNA in a gel to bind to DNA probes in order to detect the presence of particular genes or sequences of DNA. The enzyme reverse transcriptase can be used by viruses to insert viral mRNA into the host genome.

Pediatrics Best R.

12) Which of the following is a recognized treatment for complications of cystic fibrosis? Available marks are shown in brackets 1 ) DNAase to assist in reinflating collapsed lung segments. 2 ) Rectal pull-through and anastamosis for rectal prolapse. 3 ) Pancreatic transplant for diabetes mellitus. Nebulised Tobramycin for pseudomonas colonization of the lower respiratory 4) tract. 5 ) Hypotonic saline drinks for hypernatraemic dehydration.

[0] [0] [0] [100] [0]

Comments: Human recombinant DNAase given as a single daily aerosol seems to improve pulmonary function, decrease the frequency of chest exacerbations, and promotes a sense of well-being in patients with mild to moderate disease with purulent secretions. This may be because, in the inflamed airway, the nuclei from dead cells accounts for much of the viscidity of secretions. Rectal prolapse is usually idiopathic, occurring between 1 and 5 years. Intestinal parasites, malnutrition, acute diarrhoea, ulcerative colitis, pertussis, Ehler's Danlos Syndrome, meningocele, cystic fibrosis, and chronic constipation can also predispose to it. Following defecation the prolapse usually resolves spontaneously, or through manual reinsertion by the patient or parent. Nebulised Tobramycin or Gentamicin may be given when airway pathogens are resistant to oral antibiotics, or where infection is difficult to control at home. Hypernatramic dehydration should be treated in the usual way.
13) Characteristic features of physiological jaundice include all of the following except: Available marks are shown in brackets 1 ) Onset after the first 24 hours of life. 2 ) Disappearance by the tenth day of life. 3 ) A serum bilirubin level below 250 mmol/L. 4 ) Normal feeding behaviour 5 ) Associated anaemia.

[0] [0] [0] [0] [100]

Comments: Physiological jaundice occurs in 90% of infants and manifests after the first 2-3 days of life it is related to increased red cell breakdown and relative immaturity of hepatic function. Anaemia if present would imply pathological cause of jaundice eg haemolytic anaemia.

Pediatrics Best R.

14) A 3 year old by presents with a 2d history of coryza and cough, particularly at night. On examination he has Harrison's sulci and is on the 3rd centile for weight and height. Chest auscultation reveals scattered wheeze. What is the most likely diagnosis? Available marks are shown in brackets 1) Gastroesophageal reflux 2) Allergic rhinitis 3) Sinusitis 4) Asthma 5) Croup

[0] [0] [0] [100] [0]

Comments: The examination findings suggest chronic undertreated asthma. If he does not respond rapidly to asthma therapy a sweat test may be indicated to exclude CF. It is likely that he will respond to regular inhaled low-dose steroids.
15) A 4 year old girl is referred with behavioural problems. Her speech is generally wellformed. However, she finds it difficult to sustain conversations because she talks obsessively about her own interests. She avoids eye contact. She plays on her own for hours with her dolls, dressing and undressing them repeatedly. Attempts to play with others results in tantrums EFG HIJKL. Born at 39+6/40, 3.18kg no neonatal problems. No drugs, allergies. Fully immunized. No FH/SH of note. On examination she is on the 50% for height and weight. There are no specific abnormalities to find. What is the most likely diagnosis? Available marks are shown in brackets 1) Speech delay, isolated 2) Pervasive developmental disorder 3) Global developmental delay 4) Impaired hearing 5) Autism

[0] [100] [0] [0] [0]

Comments: This child has problems with reciprocal social interaction, with repetitive obsessive play. Her speech is normal in form. These symptoms are characteristic of Asperger syndrome. This is part of the spectrum of Pervasive Developmental Disorders, and is regarded by some as 'high-functioning autism'. Such children may be regarded as eccentric by their peers.

Pediatrics Best R.

16) A 9 year old boy presents with a history of headache and persistent green nasal discharge. At night he has a cough and snores loudly. The headache is exacerbated by leaning forwards. On examination he is apyrexial, but has a persistent nasal obstruction and nasal speech. He is tender over the maxillae and forehead. What is the most likely diagnosis? Available marks are shown in brackets 1) Gastroesophageal reflux 2) Allergic rhinitis 3) Sinusitis 4) Asthma 5) Croup

[0] [0] [100] [0] [0]

Comments: The picture is one of upper airways obstruction associated with nasal discharge, most likely due to sinusitis. In this case the maxillary and frontal sinuses are most likely to be involved.
17) 15 year old boy is knocked out for 2 minutes while playing rugby. He recovers rapidly, but cannot recall the incident. Apart from mild asthma requiring beta-agonists before sport he is otherwise well. On examination his temperature is 36.5C (tympanic), RR 12/min and HR 65/min. His GCS is 15/15 and no focal neurological abnormalities are present. What is the most likely diagnosis? Available marks are shown in brackets 1) Child abuse 2) Head injury 3) Hypoglycaemia 4) Diabetic ketoacidosis 5) Substance abuse

[0] [100] [0] [0] [0]

Comments: He has had a traumatic concussion, which refers to a diffuse brain injury. It is not necessary to be knocked out to have concussion. The Colorado Medical Society guidelines grade such injuries into 3 groups:
1. Confusion alone 2. Confusion and amnesia 3. Confusion, amnesia and loss of consciousness To avoid the Second Impact Syndrome return to sport should be allowed after 20 mins (group 1), 1 week (group 2) or 1 month (group 3) respectively.

Pediatrics Best R.

18) A 17 year old student presented with recurrent attacks of dizziness. Which one of the following additional features is most suggestive that she has an anxiety disorder? Available marks are shown in brackets 1) elevated diastolic blood pressure 2) nocturia 3) paraesthesiae in the hands 4) rotational vertigo 5) tinnitus

[0] [0] [100] [0] [0]

Comments: Parasthesiae is often associated with anxiety of any description. Rotational vertigo and tinnitus suggest an organic disorder, whilst anxiety disorder would normally be associated with raised systolic blood pressure 19) A young boy is presents with aches and pains. Which of the following features would argue against a diagnosis of osteomalacia? Available marks are shown in brackets 1) Bow legs 2) raised alkaline phosphatase 3) Low serum phosphate level 4) Normal serum calcium 5) advanced bone age

[0] [0] [0] [0] [100]

Comments: Clinical features include: bowing of tibia and fibula, craniotabes (soft skull), thickening of forearm at the wrist, and of the costo chondral junction (rachitic rosary). Dietary Vitamin D deficiency stimulates secondary hyperparathyroidism, which causes increased renal excretion of phosphate, low serum phosphate, and reduced urinary calcium excretion. Calcium concentrations may be low or low normal. Raised alkaline phosphatase results from osteoblasts forming unmineralised matrix. Short stature and delayed bone age are associated, rather than advanced bone age.

Pediatrics Best R.

20) A 16 year old girl presents with a 2 day history of deteriorating breathlessness and dyspnoea. Blood gas analysis shows a pH of 7.25, a PCO2 of 7.0kPa, a PO2 of 8.5kPa, and a base excess of -4. Which of the following interpretations is correct? Available marks are shown in brackets 1 ) Results are consistent with bronchopulmonary dysplasia. 2 ) Blood gases suggest type 1 respiratory failure. 3 ) Immediate intubation is required. 4 ) Results are consistent with late severe asthma. 5 ) Bicarbonate may be necessary to correct the acidosis.

[0] [0] [0] [100] [0]

Comments: In interpreting blood gas results, the following sequence may be useful:
Inspect the pH: Is it low, normal or high? Inspect the CO2: Is it low, normal or high? Inspect the PO2: Is it low, normal or high?

If the pH is low then an acidosis is present, and inspecting the CO2 will enable you to determine whether this is due to respiratory or metabolic causes. Inspecting the PO2 will tell you whether the patient is hypoxic or not. In this case, the pH is reduced, and the CO2 is high, with a base deficit of only -4, insufficient to explain the acidosis from metabolic causes. This is, therefore, a respiratory acidosis, and the PO2 is also low suggesting type 2 respiratory failure. Possible causes would include severe pneumonia, end stage asthma or neurogenic causes such as guillain-Barre. In asthma, the initial stages show a low CO2, with this climbing only to accompany failing respiration. The results would therefore be consistent with late severe asthma. In bronchopulmonary dysplasia, there is usually long-term CO2 retention with compensatory increase in bicarbonate leading to a positive base excess and normal pH. Bicarbonate is usually only considered if the base deficit exceeds about -8.

Pediatrics Best R.

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21) An 18 month old boy because of maternal concerns about delayed speech. He was born at 39+4/40 weighing 2.6kg and there were no neonatal problems. He sat at 12/12, and now pulls to stand. He is able to make a tower of 2 inch-high bricks. He is saying 23 single words which his mother can understand and seems to hear well. He drinks from a bottle. He is fully immunized, on no medications, and there is no FH/SH of note. On examination he looks well. He is on the 25th centile for height and weight and OFC. There are no specific findings of note. What is the most likely diagnosis? Available marks are shown in brackets 1) Speech delay, isolated 2) Pervasive developmental disorder 3) Global developmental delay 4) Impaired hearing 5) Autism

[0] [0] [100] [0] [0]

Comments: The history suggests moderate global developmental delay. He is functioning at about the 12 month level in gross motor, fine motor and vision, hearing and speech, and social development. The history gives no indication of an underlying cause for the delay. A detailed developmental assessment should be performed plus investigations for an underlying cause. At minimum these should include TFTs and karyotype to rule out Fragile X Syndrome. Other investigations may be indicated if a specific disorder is suspected.
22) A 15 month old girl presents with stridor and respiratory distress. She has had a crusty nasal discharge for 2 days with low grade fever. She went to bed as usual at 8pm, but awoke 2 hours later. Previous history was unremarkable. On examination she has a fever of 37.8C (tympanic), RR of 55/min (crying), and HR of 150/min. She has marked stridor with moderate recession and a barking cough. O2 saturations are 95% in air. What is the most likely diagnosis? Available marks are shown in brackets 1) Retropharyngeal abscess 2) Foreign body aspiration 3) Anaphylaxis 4) Croup 5) Peritonsillar abscess

[0] [0] [0] [100] [0]

Comments: The history suggests a viral croup (laryngotracheobronchitis). Oral dexamethasone or inhaled budesonide are effective in reducing symptoms, but are not required in mild cases. Reassurance of the mother often calms down both child and mother.
Pediatrics Best R.

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23) A 14 year old girl presents with a history of cough and breathlessness on exercise. She has seasonal rhinitis, and admits to have started smoking. Clinical examination is unremarkable. What is the most likely diagnosis? Available marks are shown in brackets 1) Gastroesophageal reflux 2) Allergic rhinitis 3) Sinusitis 4) Asthma 5) Croup

[0] [0] [0] [100] [0]

Comments: A typical history of asthma in later childhood, with exercise-induced symptoms and a general deterioration on commencement of smoking. Unfortunately this is all too common these days.
24) A 10 year old boy attends A+E with a two day history of a limp. He has recently been unwell. On examination he is apyrexial and movement at his hip is uncomfortable. Available marks are shown in brackets 1) Congenetal dislocation. 2) Perthes disease 3) Septic arthritis 4) Slipped upper femoral epiphesis 5) transient synovitis

[0] [0] [0] [0] [100]

Comments: Transient synovitis is the most common cause of hip pain in childhood but other more serious causes should be excluded before it is diagnosed. The cause is unknown but it can be related to viral infection, allergic reaction or trauma.

Pediatrics Best R.

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25) A 9 week old infant is brought in unresponsive. Mother says she has well apart from episodes of screaming every evening, which last several hours at a time. She was born at 38+2/40 weighing 3.1kg and there were no neonatal problems. Parents are unmarried teenagers. On examination she has 3 small possible bruises on her face. She has a temperature of 34.5C, RR 30/min (irregular pattern), HR 130/min. She is floppy with a full fontanelle. Fundoscopy shows flame-shaped haemorrhages. BM stix is 3.9 mmol/l. What is the most likely diagnosis? Available marks are shown in brackets 1) Child abuse 2) Hepatic failure 3) Hypoglycaemia 4) Diabetic ketoacidosis 5) Substance abuse

[100] [0] [0] [0] [0]

Comments: The history suggests a previously healthy child with colic of inexperienced parents. The facial bruising suggests grip marks. Given the full fontanelle, comatose state of the child, and flame-shaped haemorrhages, Shaken baby syndrome is most likely. It is important to exclude infection with DIC or a coagulopathy.
26) A 2 year old child presents with delayed language development. He was born at term weighing 3.21kg and there were no neonatal problems. Gross motor, vision and fine motor development are normal. Mother complains that he 'doesn't listen' especially when he is in another room. His speech is restricted to single words, and only mother understands them. On examination he is apyrexial, RR 16/min, HR 90/min. No dysmorphic features. Chest is clear, there is no murmur nor organomegaly. ENT examination reveals normal tonsils, clear nose and bilaterally dull drums. What is the most likely diagnosis?<> Available marks are shown in brackets 1) Speech delay, isolated 2) Pervasive developmental disorder 3) Global developmental delay 4) Impaired hearing 5) Autism

[0] [0] [0] [100] [0]

Comments: The child has hearing impairment and isolated language delay. The examination suggests bilateral 'glue ear' (Otitis media with effusion). This usually resolves with time, though it may require tympanostomy tube insertion. These children often need Speech Therapy input.

Pediatrics Best R.

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27) A 3 year old girl presents with a 5 day history of neck pain and fever, with increasing difficulty in swallowing. Over the previous night she woke several times with difficulty in breathing. She was born at 36+6/40 weighing 2.8kg and there were no neonatal problems. On examination her head held to the right. She has audible stridor when agitated, and she has very tender glands in the neck. Her temperature is 38.6C, RR 20/min and HR 100/min. She has moderate neck stiffness. What is the most likely diagnosis? Available marks are shown in brackets 1) Retropharyngeal abscess 2) Foreign body aspiration 3) Anaphylaxis 4) Croup 5) Peritonsillar abscess

[100] [0] [0] [0] [0]

Comments: The history suggests a tonsillitis progressing to retropharyngeal abscess with increasing upper airways obstruction. ENT examination in children with stridor should take place where facilities are available for immediate intubation. In this case it revealed a massively enlarged right tonsil with profuse exudate. This required surgical drainage and removal with antibiotic cover.
28) A 2.5 year old boy is referred with behaviour problems and poor speech. He speaks very seldom, and is unable to sustain a conversation. He always prefers to play on his own, lining up his Thomas the Tank engine set repetitively, ignoring his older and younger siblings. He throws tantrums when his routine is disturned. He was born at 37+3/40 weighing 3.51kg and there were no neonatal problems. Pregnancy had been uncomplicated. On examination he is apyrexial, and there are no specific abnormalities to find. What is the most likely diagnosis? Available marks are shown in brackets 1) Speech delay, isolated 2) Pervasive developmental disorder 3) Global developmental delay 4) Impaired hearing 5) Autism

[0] [0] [0] [0] [100]

Comments: The history is of poor speech development, ritualistic behaviour and lack of reciprocal social interaction. The most likely diagnosis is Autism, which starts before 30 months of age and is characterized by a qualitative impairment in verbal and nonverbal communication, in imaginative activity, and in reciprocal social interactions. It is part of the wider spectrum of pervasive developmental disorders. This includes: autism, Aspergers, childhood disintegrative disorder, and Rett's syndrome.

Pediatrics Best R.

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29) A 12 year old boy complains of persistent clear nasal discharge during the spring and summer. He constantly rubs his nose and his eyes. Sometimes he has an associated cough. The symptoms usually get better in the autumn. He was diagnosed with asthma at 2 years, but this has improved with age. He rarely requires beta agonist inhaler. On examination he has a clear nasal discharge, nasal speech, and pink non-purulent conjunctivae. What is the most likely diagnosis? Available marks are shown in brackets 1) Gastroesophageal reflux 2) Allergic rhinitis 3) Sinusitis 4) Asthma 5) Croup

[0] [100] [0] [0] [0]

Comments: This atopic boy has classical symptoms of seasonal rhinitis. Although skin prick testing may be helpful in resistant cases, it is usual to treat sufferers symptomatically with oral antihistamines or topical steroids to eyes and nose.
30) 5 month old boy has had a mild coryza for 2 days. Mother hears him making odd noises on the baby monitor. When she investigates she finds him floppy, pale and not breathing. She stimulates him, attempts mouth-to-mouth resuscitation and calls an ambulance. They give bag ventilation and he starts breathing again. O2 is given on the way to hospital. Born at 32/40 gestation weighing 1.7kg he required 3d ventilation for surfactant-deficient lung disease. Since discharge he has been thriving. On arrival at hospital he is self-ventilating in facemask oxygen, with saturations of 94%. Temperature is 37.8C, with RR 35/min, mild recession, and HR of 140/min. Scattered coarse crepitations are audible in both lung fields. What is the most likely diagnosis? Available marks are shown in brackets 1) Acute life-threatening event 2) Cardiac dysrhythmias 3) Seizures 4) Sudden infant death syndrome

[100] [0] [0] [0]

Comments: This child has RTI followed by acute life-threatening event (ALTE). There are a large number of potential causes. In this case bronchiolitis is most likely, given the examination findings.

Pediatrics Best R.

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31) A 3 year old child presents with delayed speech. Although he seems to understand full sentences and commands he says only occasional single words. He was born at 41/40 weighing 3.4kg and there were no neonatal problems. He is fully immunised, on no medications, and comes from a middle class articulate family. On examination he is on the 25th centile for height, weight and OFC. There are no specific abnormalities to find. ENT examination is unremarkable. What is the most likely diagnosis? Available marks are shown in brackets 1) Speech delay, isolated 2) Pervasive developmental disorder 3) Global developmental delay 4) Impaired hearing 5) Autism

[100] [0] [0] [0] [0]

Comments: The history is of isolated speech delay in a child who apparently hears normally. Hearing should be formally checked, as it is the commonest reason for speech delay.In this case the problem involves mainly expression rather than reception of speech. Such speech problems may interfere significantly with schooling.
32) The parents of an eight year child divorce. The child lives with his mother with his father visiting at weekends. On his father's visits, the child is sullen and irritable but when his father leaves the boy cries, clings to his father and askes his father to come back to live with his mother. What is the most appropriate response that the father can make during these episodes? Available marks are shown in brackets 1 ) Big boys don't cry 2 ) I left your mother but I am not leaving you. 3 ) I will see you next weekend 4 ) You take care of the house now I've left 5 ) Sorry son but I just couldn't live with your mother

[0] [100] [0] [0] [0]

Comments: The child's reactions typically represent a feeling of abandonment and it is important that his father reaffirms that he is not leaving his son.

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16

33) An infant is diagnosed with pyloric stenosis. Which of the following is true of this diagnosis? Available marks are shown in brackets 1 ) Typically presents between 6 and 12 months of age. 2 ) Is more common in females. 3 ) There is a strong familial predisposition. 4 ) Is due to hypertrophy of the longitudinal muscle layer of the pylorus. 5 ) Presents with bile-stained projectile vomiting.

[0] [0] [100] [0] [0]

Comments: Infantile hypertrophic pyloric stenosis presents most commonly at approximately 6 weeks of age. It is more common in boys with a male: female ratio of 4:1. There is a strong familial predisposition with 5% of affected infants having a mother who also developed the problem. Pathologically, the pyloric 'tumour' arises from hypertrophy the circular muscle layer. Patients present with projectile vomiting that is not bile stained. Following resusciation and correction of any electrolyte abnormality, surgical treatment is by pyloromyotomy. 34) A study has been designed to investigate whether a certain drug plus physiotherapy treatment is better than drug treatment alone in the management of rheumatoid arthritis. After randomizing the patients a small proportion of the drug plus physiotherapy group decide to drop out of the study or omit some treatment sessions specified in the research protocol. What is the correct way of analysing the subsequent data? Available marks are shown in brackets 1 ) Assume the patients have withdrawn their consent 2 ) Exclude these patients from all analysis 3 ) Extend the trial recruitment to make up the numbers 4 ) Include these patient outcomes in the drug plus physiotherapy group 5 ) Interview the patients and report their group separately

[0] [0] [0] [100] [0]

Comments: This is the principle of 'intention to treat'. It is possible that the physiotherapy intervention was harmful to the patients and this is why they left. Intention to treat helps to reduce bias by sticking to the original allocation of treatment and analyzing the patient in that treatment group even (and concentrate for this bit) even if they don''t get it!

Pediatrics Best R.

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35) A 17 year old female presents with acute breathlessness. She has had asthma for approximately 3 years and recently commenced new therapy. Which agent may be responsible for this exacerbation? Available marks are shown in brackets 1) Salmeterol 2) Theophylline 3) Beclomethasone 4) Ipratropium bromide 5) Monteleukast

[100] [0] [0] [0] [0]

Comments: Salmeterol has been reported to produce an acute exacerbation of asthma, possibly through an acute hypersensitivity reaction. 36) A 17 year old male is brought to clinic as his parents are concerned about changes in his behaviour. Which of the following suggest a diagnosis of Schizophrenia? Available marks are shown in brackets 1 ) incongruity of affect RSNTVWX YMNOZ KLMNOPQ RSNTQ 2 ) auditory hallucinations with clouding of consciousness 3 ) memory impairment 4 ) feelings of panic in buses and shops 5 ) grandiose ideations

[100] [0] [0] [0] [0]

Comments: Incongruity of affect is emotion inappropriate to circumstances. There may be intellectual defects from prolonged institutionization [N\N]WX ^_` [NabcVL KQNdeX or treatment rather than the illness itself. Auditory hallucinations with clouding of conciousness suggest delirium and memory impairment possible organic brain disease. The panic in crowds suggests an anxiety disorder and grandiose ideations suggest hypomania 37) An 18 year old female is reluctant to eat food that is prepared for her. Which one of the following would be most consistent with a diagnosis of anorexia nervosa? Available marks are shown in brackets [0] 1) she believes the food is poisoned 2) she has a full-time job [100] 3) she has bouts of heavy drinking [0] 4) she regards herself as ill [0] 5) she secretly abuses anabolic steroids [0] Comments: Anorexia nervosa is associated with the abnormal perception of body image. They generally feel well despite the protestations of others who feel that they look awful. They exercise avidly and until the very late stages of the disease hold down full time jobs. There is no delusion with regard to the food being poisoned. Heavy drinking associated with food refusal would suggest alcoholism and alcoholic gastritis. The secretive abuse of laxatives would fit with the diagnosis rather than anabolic agents.

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38) Which of the following organelles contains enzymes responsible for the digestion of constituents of cells and tissues? Available marks are shown in brackets 1) endoplasmic reticulum 2) Gogli apparatus 3) lysosomes 4) microtubules 5) mitochondria

[0] [0] [100] [0] [0]

Comments: The lysosomes contain the enzymes and molecules such as oxidases, free radical etc responsible for the breakdown of intracellular components. Micortubules are involved in mitotic processes and intracellular transportation. The mitochondria produce energy for cellular functions.
39) Which one of the following is true regarding protein metabolism? Available marks are shown in brackets 1 ) There are 5 essential amino acids. The absence of any of the essential amino acids results in a negative nitrogen 2) balance Protein constitutes about 30-45% of total daily calorie intake in infants and 3) children 4 ) Essential amino acids can be synthesized in the liver and kidneys Human milk as the only food provides an insufficient source of protein for a 1 5) year old healthy infant

[0] [100] [0] [0] [0]

Comments: There are 9 essential amino acids; all are required to maintain nitrogen balance. Carbohydrates constitute 30-45% of total caloric intake in children. Essential amino acids cannot be synthesized and must be obtained from the diet. Human milk contains the proteins casein, lactoglobulin and lactalbumin, which provide a sufficient source of protein.

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40) Apoptosis is the process of programmed cell death and occurs in cells that have damaged DNA. A mediator of this process is a tumour suppressor gene that inhibits mitosis and promotes apoptosis. This gene is:Available marks are shown in brackets 1) bcl-2 2) caspases 3) fas (CD95) 4) p53 5) ras

[0] [0] [0] [100] [0]

Comments: bcl-2 is an inhibitor of apoptosis. fas is a cell receptor and caspases are present in all cells both promote apoptosis but are not tumour suppressor genes. ras is an oncogene.
Which one of the following conditions is transmitted by means of an X-linked recessive inheritance : Available marks are shown in brackets 1) 2) 3) 4) 5) Cystic fibrosis Galactosaemia Huntingtons chorea Duchenne muscular dystrophy Facio-humero-scapular dystrophy [0] [0] [0] [100] [0]

Comments: Cystic fibrosis and Galactossaemia are autosomal recessively inherited conditions. Haemophilia and Duchenne muscular dystrophy are X- linked recessive and Facio-humero-scapular dystrophy and Huntingtons chorea are inherited in an autosomal dominant manner.

A 10 year old child is found to have neurofibromatosis. Which of the following statements correctly applies: Available marks are shown in brackets 1) 2) 3) 4) 5) She will most probably be mentally retarded The finding of 2 caf au lait spots in her 6 year old sibling suggests he may be affected Her condition was inherited in an autosomal recessive fashion Freckling of the back would be expected There may be no family history of the condition [0] [0] [0] [0] [100]

Comments: Mental retardation may be a clinical feature of the condition but is not a usual clinical feature. Six or more caf au lait spots before the age of puberty would assist in making a diagnosis, along with other clinical findings such as Lisch nodules, and optic nerve gliomas. The condition is autosomal dominant. Freckling of the inguinal and axillary regions occur. 30-50% of new cases are due to spontaneous mutations.

Which of the following statements applies to fragile X syndrome?

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Available marks are shown in brackets 1) 2) 3) 4) 5) Mental retardation is found in all males Mental retardation is not found in females Cytogenetic finding of fragile-X is induced in a vitamin B12 deficiency culture medium Affected children are taller than average The condition can only be diagnosed after birth. [0] [0] [0] [100] [0]

Comments: Affected males are usually but not always mentally retarded .One third of females with the mutation will be mentally retarded. The diagnosis of fragile X syndrome was originally based on the expression of a folate- sensitive fragile X site (X q27.3) induced in cell culture under conditions of folate deprivation. Affected children are taller and may have high arched palate, long ears, a long face and macro orchidism. Diagnosis can be made by detection of the mutant FMR 1 gene by chorionic villus sampling , confirmatory amniocentesis may be required in some cases.

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