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Royal College of Paediatrics and Child Health


MRCPCH PART I EXAMINATION PAPER One B Specimen Paper
1. Complete the following: Your full name (BLOCK LETTERS) RCPCH Number. Signature 2. Check your surname (family name) and initials appear in the top left hand corner of the Answer Sheet. Check your candidate number is in the top right hand corner. Using the pencil provided, complete your response to each item on the Answer Sheet. 3. It is strictly forbidden to talk to, read the work of, or attempt in any way to communicate with, other candidates whilst the examination is in progress. Please exercise vigilance to ensure that no other candidate can attempt to copy your work. The College has tools which can identify copying of answers or collusion between candidates to share answers. In any situation the suspicion of guilt falls upon both parties until it can be proved otherwise. Breaches of these instructions, or misbehaviour in any other way, including continuing to write after the allotted time, may lead to suspension from the examination at the discretion of the invigilators. Serious breaches, such as cheating or colluding to gain advantage, could incur permanent suspension from College examinations. 4. Question papers and individual questions must not be copied or removed from the examination room. 5. Copyright law protects examination questions and the intellectual property of their authors. The unauthorised use of questions is a breach of copyright law. 6. Answer all the following 75 questions.

EXTENDED MATCHING QUESTIONS Qu 1 This is a list of genetic mechanisms: A B C D E F G H I J Anticipation Autosomal dominant Autosomal recessive Chromosomal Isodisomy Microdeletion Mitochondrial X-linked dominant X-linked recessive Y-linked

Choose the most likely method of genetic inheritance for each of the following: SELECT ONE ANSWER ONLY FOR EACH QUESTION Note: Each answer may be used more than once 1. A 5-year-old boy presents with attention deficit hyperactivity disorder. As an infant he had interrupted aortic arch syndrome and a cleft lip both of which have been repaired.

2. A baby girl is noted on the post-natal ward to have the following: Low birth weight; a coloboma; loud, radiating systolic murmur; cleft palate, low set ears; flexed fingers.

3. A 6-month-old boy is referred by the Health Visitor because the head th circumference is persistently at the 97 centile. He weighed 3.9kg at term th and his weight is on the 75 centile. His development is normal.

Qu 2 This is a list of diagnoses: A B C D E F G H I J Acute lymphoblastic leukaemia Developmental dysplasia of the hip Growing pains Juvenile idiopathic arthritis Non-accidental injury Osteomyelitis Perthes disease Reactive arthritis Rickets Slipped upper femoral epiphysis

Select the most likely diagnosis for each of the following: SELECT ONE ANSWER ONLY FOR EACH QUESTION Note: Each answer may be used more than once 1. A 12-year-old girl of Caribbean origin presents with pain in the thigh and a limp. She has been well in the past apart from occasional colds. There is no evidence of swelling in the joints but internal rotation and abduction are limited. She is well-grown for her age and in mid-puberty. The pain started after a netball match.

2. A 5-year-old girl of Caucasian origin presents with limp and swelling of the knee but little complaint of pain. On examination there is muscle wasting and some limitation of movement. The joint is slightly warm to touch and there is peri-articular joint swelling and a small joint effusion.

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Question 2 continued 3. A 3-year-old boy presents with a history of pain in his left hip for last 2 days. Today he is reluctant but able to walk if coaxed. He is afebrile. There is restricted movement of his left hip joint. He is on trimethoprim prophylaxis for recurrent urinary tract infections associated with left vesico-ureteric reflux. He has recently recovered from a chest infection treated with amoxicillin.

Qu 3 This is a list of management options: A B C D E F G H I J Defibrillation Ice pack on the face Inhaled salbutamol Intravenous adenosine Intravenous amiodarone Intravenous lignocaine Intravenous sotalol Oral digoxin Radiofrequency ablation Synchronised DC cardioversion

Choose the best treatment for each of the following: SELECT ONE ANSWER ONLY FOR EACH QUESTION Note: Each answer may be used more than once 1. An overweight 3-month-old presenting with narrow complex tachycardia at 300 beats/min.

2. An unconscious 4-year-old found drowned in a pond, with ventricular fibrillation. 3. Recurrent episodes of SVT in a 7-year-old, despite treatment with 3 different anti-arrhythmic drugs over 4 years.

Qu 4 This is a list of management options A B C D E F G H I J Carbamazepine Clonazepam Diazepam Ethosuximide Explanation and reassurance Gabapentin Ketogenic diet Lamotrigine Lorazepam Vigabatrin

Choose the best management for each of the following: SELECT ONE ANSWER ONLY FOR EACH QUESTION Note: Each answer may be used more than once 1. A 7-year-old-boy was thought to be day-dreaming in class. have generalised absence seizures. He is found to

2. A 2-year-old boy has recurrent attacks when he goes pale and falls to the ground after minor trauma to the head. 3. An 18-month-old girl is admitted following a 5-minute generalised convulsion associated with a high fever. She had a similar episode 6 months ago.

Qu 5 This is a list of organisms: A B C D E F G H I J Coagulase negative staphylococcus Enterococci Group A streptococcus Group B streptococcus Methicillin resistant staphylococcus aureus Proteus vulgaris Staphylococcus aureus Streptococcus faecalis Streptococcus pneumoniae Streptococcus viridans

Choose the organism which matches most closely each of the following:: SELECT ONE ANSWER ONLY FOR EACH QUESTION Note: Each answer may be used more than once 1. A 4-week-old boy was born with spina bifida and hydrocephalus. The spinal lesion was closed shortly after birth and a ventricular peritoneal shunt was inserted a week ago. He has a temperature of 37.8C.

2. 4-year-old boy is admitted with right-sided pyelonephritis. X-ray demonstrates renal stones. 3. A previously fit 5-year-old-girl is just recovering from chicken pox. She presents with a swollen, painful red area on her right shin and is refusing to walk. Bone scan shows increased uptake in the right tibia.

Qu 6 This is a list of diagnoses: A B C D E F G H I J Choanal atresia Chronic sinusitis Laryngeal nerve palsy Laryngeal papillomatosis Laryngomalacia Obstructive sleep apnoea Subglottic stenosis Tracheal haemangioma Tracheoesophageal fistula Vascular ring

Choose the most likely diagnosis for the following: SELECT ONE ANSWER ONLY FOR EACH QUESTION Note: Each answer may be used more than once 1. A 9-year-old boy is evaluated for with poor concentration and difficult behaviour at school. He suffers from recurrent upper respiratory tract infections, not helped by tonsillectomy a year ago.

2. A 4-year-old girl has occasional stridor. She has difficulty swallowing lumpy food. 3. A girl is born at term and cries immediately. She then develops significantly increased work of breathing and needs to be ventilated. Extubation is unsuccessful on 3 occasions.

Qu 7 This is a list of diagnoses: A B C D E F G H I J ABO Incompatibility Biliary atresia Breast milk jaundice Galactosaemia Glucose-6-phosphate dehydrogenase deficiency Hypernatraemic dehydration Neonatal hepatitis Physiological jaundice Rhesus Incompatibility Sepsis

Choose the most likely diagnosis for the following: SELECT ONE ANSWER ONLY FOR EACH QUESTION Note: Each answer may be used more than once 1. A baby boy was born at term weighing 3.6Kg and is breast-fed. At 5 days of age he is admitted with poor feeding, drowsiness and jaundice. His weight is 3.06K. Mothers blood group A positive Babys blood group haemoglobin

A positive 17 g/dl

bilirubin 250 ml/l (10 ml/l conjugated) urea 11.0 mmol/l sodium 151 mmol/l potassium 3.6 mmol/l C-reactive protein (CRP) 10 mg/l

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Question 7 continued 2. A baby boy was born at term, to Turkish parents, weighing 3.65Kg. He was discharged home bottle-feeding well at 2 days. He presents at 5 days of age clinically jaundiced. He looks well, weight 3.5 Kg Mothers blood group A positive Babys blood group haemoglobin

O positive 11.5 g/dl

bilirubin 400 mol/l (200 ml/l conjugated) urea 4.2 mmol/l sodium 137 mmol/l potassium 3.6 mmol/l C-reactive protein (CRP) 9.5 mg/l 3. A baby boy was born at term weighing 3.47Kg. His mother wished to take him home and he was discharged breast-feeding well. Routine neonatal examination was normal. He presents at 6 days of age clinically jaundiced and feeding less well. His weight is 3.4 Kg. Clinical examination is normal except that the red reflexes are not elicited. Mothers blood group O positive Babys blood group haemoglobin bilirubin urea sodium potassium C-reactive protein (CRP)

O positive 14.5 g/dl 300 mol/l (50 ml/l conjugated) 6.2 mmol/l 137 mmol/l 3.6 mmol/l 13.5 mg/l

Qu 8 This is a list of diagnoses: A B C D E F G H I J Cat-scratch disease Enterovirus infection Infectious mononucleosis Kawasaki disease Lyme disease Measles Necrotising fasciitis Streptococcal pharyngitis Toxic shock syndrome Typhoid fever

Choose the most likely diagnosis for each of these children presenting with fever: FOR SELECT ONE ANSWER ONLY FOR EACH QUESTION Note: Each answer may be used more than once 1. An 8-year-old boy presents with a 3-day history of fever and sore throat. He is febrile (38.8C), his tonsils are covered in exudate and he has cervical and axillary lymphadenopathy. His pulse is 160/min and there is a grade 2/6 systolic murmur heard over the left upper sternal edge. There is a faint macular rash over his trunk and enlargement of the liver and spleen.

2. A 17-month-old boy presents with a 4-day history of fever, cough, runny nose and red eyes. He is febrile (38.8C) and miserable. He has a non-specific macular rash on the body. He has a red throat with slight tonsillar enlargement. One cervical lymph node is enlarged (1.5 x 2 cm) and tender. His lips, hands feet are red. There is no peeling of the fingers. 3. An 18-month-old boy presents with a 3-day history of fever and coryzal symptoms. He has decreased movements of his right upper limb over the past 24 hours. He is febrile (38.8C) and has a faint macular rash over his trunk. He has flaccid paralysis of his right upper limb and the biceps, triceps and brachioradialis jerks cannot be elicited. He is conscious, alert and has no signs of meningism.

Qu 9 This is a list of causes of gastrointestinal bleeding: A B C D E F G H I J Anal fissure Angiodysplasia Colonic polyp Cows milk allergy Crohns disease Diverticulitis Haemolytic uraemic syndrome Henoch Schnlein purpura Sexual abuse Shigella enteritis

Choose the most likely diagnosis for each of the following: SELECT ONE ANSWER ONLY FOR EACH QUESTION Note: Each answer may be used more than once 1. A 3-year-old boy presents with a 2-day history of bloody diarrhoea and fever. He is clinically dehydrated and toxic. He has a non-blanching petechial rash on his trunk. Blood haemoglobin 7.0 g/dl 9 white cell count 15.1 x 10 /l 9 platelet count 20 x 10 /l albumin 38 g/l

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continued Question 9 continued

2. An 8-year-old boy presents with a 6-month history of bloody diarrhoea associated with weight loss and lethargy. He is pale but there are no diagnostic clinical features. Blood haemoglobin 9.0 g/dl 9 white cell count 10.1 x 10 /l 9 platelet count 524 x 10 /l albumin 28 g/l

3. A 12-year-old boy presents with intermittent episodes of blood in his otherwise normal stools. He is well and there are no diagnostic clinical features. Blood haemoglobin 11.0 g/dl 9 white cell count 7.1 x 10 /l 9 platelet count 236 x 10 /l albumin 38 g/l

Qu 10 This is a list of investigations: A B C D E F G H I J Bronchoscopy Full blood count and film High resolution CT scan of chest Immunoglobulin E level Immunoglobulin G subsets Oesophageal pH study Peak expiratory flow reading Pulmonary function tests Short synachthen test Ultrasound scan of chest

Choose the most important way to assess the following: SELECT ONE ANSWER ONLY FOR EACH QUESTION Note: Each answer may be used more than once 1. An 8-year-old boy is taking 600 micrograms/day of inhaled fluticasone proprionate. He has poorly controlled cough and in the last week has become very lethargic after a short-lived episode of vomiting. Other children in his class had a similar illness but were less severely affected. He th has finger clubbing. His height is on the 25 centile.

2. A 2-year-old boy has been admitted to hospital 4 times in the last 6 months with a diagnosis of croup. On one occasion he required intubation and ventilation. He was born at 26 weeks gestation and was oxygen dependent till the age of 4 months. 3. A 9-year-old girl presents with a 10-day history of fever and cough. She had responded initially to oral amoxicillin but now looks ill and has stony dullness at the right base.

Qu 11 This is a list of diagnoses: A B C D E F G H I J Acute lymphatic leukaemia 2 thalassemia Glucose- 6-phosphate dehydrogenase deficiency HbE/ thalassaemia major Hepatitis B Hereditary spherocytosis Immune thrombocytopaenia (ITP) Infective endocarditis Malaria Sickle cell anaemia

Choose the most likely diagnosis for each of the following scenarios: SELECT ONE ANSWER ONLY FOR EACH QUESTION Note: Each answer may be used more than once 1. A 7-year-old boy of Indian origin, is noted to be slightly jaundiced on arrival in Great Britain. His liver is 2 cm below the right costal margin, spleen is palpable 5 cm below the left costal margin. His Hb is 6.2 g/dl.

2. A 4-year-old boy of West African origin is noted to be slightly jaundiced on arrival in Great Britain. His spleen is palpable 5 cm below the left costal margin. His Hb is 6.2 g/dl. 3. An otherwise well 7-year-old boy of Mediterranean origin becomes jaundiced during an upper respiratory tract infection. He had been treated with phototherapy in the neonatal period. His spleen is palpable 1 cm below the left costal margin. His Hb is 7.2 g/dl.

Qu 12 This is a list of diagnoses: A B C D E F G H I J Anorexia nervosa Bulimia nervosa Cannabis abuse Chronic fatigue syndrome Conduct disorder Crohns disease Diabetes mellitus Glandular fever Lymphoma Thymoma

Choose the most likely diagnosis for the following: SELECT ONE ANSWER ONLY FOR EACH QUESTION Note: Each answer may be used more than once 1. A 14-year-old boy is refusing to eat meals with the family and says he is not hungry and goes off to his room. He has plenty of friends at school. However his mother has always found him difficult to handle especially since she remarried a year ago His mother is concerned he has lost weight but he refuses to be weighed. On occasion his mother has found his underpants to be soiled and possibly blood-stained. He refuses to discuss this with his mother.

2. A 14-year-old boy is refusing to eat meals with the family. Over the last 10 days he says he is having difficulty swallowing food. He has plenty of friends at school but is having difficulty with physical education due to coughing. His mother has always found him difficult to handle especially since she re-married a year ago. She thinks he looks pale and has heard him breathing heavily in the night but when this is mentioned he rushes off to his room. He wears baggy trousers and refuses to be weighed.

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Question 12 continued

3. A 14-year-old boy is refusing to eat meals with the family but drinks large amounts of water. He enjoys sporting activities with his friends at school. However his singleparent mother had always found him difficult to handle compared with his younger sisters. When the question of meals is mentioned at home he rushes off to his room. He wears baggy trousers and refuses to be weighed, insisting that he is all right.

MULTIPLE TRUE/FALSE QUESTIONS

Qu 13 Erythema nodosum A. characteristically leaves scarred areas after resolution B. consists of painless subcutaneous nodules on extensor surfaces C. has a recognized association with inflammatory bowel disease D. is a complication of poorly controlled insulin dependent diabetes mellitus E. is associated with the use of oral contraceptives

Qu 14 Preterm babies who are fed exclusively with breast milk are more likely than those fed with preterm formula to have A. a heavier body weight at 6 months B. E-coli gastroenteritis at 3 months C. necrotising enterocolitis in the neonatal period D. vitamin K deficiency E. nutritional rickets

Qu 15 Cataracts are a recognised feature of A. Down syndrome B. hyperthyroidism C. myotonic dystrophy D. toxoplasmosis E. Williams syndrome

Qu 16 In sexual differentiation A. gonadectomy is indicated in androgen insensitivity syndrome (testicular feminization ) after puberty B. hydrocolpos results from an anomaly of the Mullerian system C. the commonest cause of ambiguous genitalia in a 46XY baby is 21-hydroxylase deficiency D. the karyotype is the most appropriate guide to gender assignment in a baby with ambiguous genitalia E. ultrasound is the most appropriate initial investigation in a baby with bilateral undescended testes

Qu 17 With regards to the recreational drug Ecstasy, A. acute toxicity is potentially fatal B. it induces release of serotonin from nerve terminals C. it is an amphetamine derivative D. it may be taken legally by persons aged over 18-years E. there is clear evidence that it causes long-term brain damage

Qu 18 The following results favour the diagnosis of pre-renal failure rather than renal failure in a 3-year-old child: A. Fractional excretion of sodium of 0.8% (low) B. Urine / plasma os molality of 0.9 (low) C. Urine / plasma urea ratio of 15 (high) D. Urine osmolality of 290 mOsm/l E. Urine sodium of 50 mmol/l

Qu 19 Analysis of a sample of amniotic fluid is used to A. detect congenital hepatitis B infection B. detect duodenal atresia C. detect Potters syndrome D. predict lung maturity E. predict the severity of rhesus haemolytic disease

Qu 20 Regarding the spinal cord, A. lesions of the cortico-spinal fibres result in ipsilateral defects B. nearly all sensory information is carried by the dorsal columns C. the lower end (conus medullaris) is at about L5 in term neonates D. the pyramidal tract becomes progressively larger from above downwards E. trauma to the cervical cord typically causes urinary retention

Qu 21 Which of the following statements about mitochondria are true? A. Defects of mitochondria disrupt oxidative phosphorylation B. Mitochondria are attached to the Golgi apparatus C. Mitochondria proliferate in response to energy requirements D. Mitochondrial genes are heteroplasmic E. Mitochondrial genes are transmitted equally in both egg and sperm

Qu 22 A childs chances of developing a major behavioural problem are increased by A. being cared for by a child-minder during normal working hours B. delayed language development C. having a sibling with cerebral palsy D.inconsistent parental discipline E. masturbation

Qu 23 The following are true: A. Breast milk IgA is absorbed by the infant up to 6-months of age B. Children with IgA deficiency are at risk of recurrent meningitis C. IgM is the first immunoglobulin to be made by the fetus D. Levels of C3 complement decrease after streptococcal nephritis E. The commonest mode of inheritance of agammaglobulinemia is autosomal dominant

Qu 24 Clinically relevant adverse drug interactions occur between A. Cimetidine and oral contraceptives B. Erythromycin and carbamazepine C. Fucidic acid and flucloxacillin D. Intravenous glucose and potassium salts E. Lamotrigine and sodium valproate

Qu 25 Hypokalaemia is characteristic of A. Bartters syndrome B. congenital adrenal hyperplasia (21-hydroxylase deficiency) C. cystinuria D. pyloric stenosis E. type 1 (distal) renal tubular acidosis

Qu 26 Bronchiectasis is a complication of A. achalasia of the cardia B. coarctation of the aorta C. gastro-oesophageal reflux D. measles E. pertussis

Qu 27 Factors associated with an increased Perinatal Mortality Rate include A. assisted conception by artificial insemination with donor sperm B. maternal short stature C. paternal age D. residence in inner cities E. third pregnancies

BEST OF FIVE QUESTIONS

Qu 28 A baby boy was clinically normal at the neonatal examination. At the age of 10-weeks he is taken to the emergency department because of persistent crying. He is cyanosed and screaming as if in pain. What is the most likely diagnosis? SELECT ONE ANSWER ONLY A. Anomalous insertion of the coronary arteries B. Hypoplastic left heart syndrome C. Tetralogy of Fallot D. Total anomalous pulmonary venous drainage E. Transposition of the great arteries

Qu 29 A 4-month-old male baby has been irritable and interacting poorly with his mother for a few weeks. During this time, he was said to be colicky because he would often jerk forward and cry. He has been vomiting 2 or 3 times daily and has not passed a stool for 3 days. What is the most appropriate investigation to arrange? SELECT SELECT ONE ANSWER ONLY A. Abdominal ultrasound examination B. Barium swallow C. EEG D. pH monitoring study E. RAST for cows' milk allergy

Qu 30 Routine antenatal ultrasound scan at 20 weeks reveals an enlarged bladder and kidneys in a male fetus. A provisional diagnosis of posterior urethral valves is made. If the mother delivers spontaneously at 33 weeks gestation, which is the complication most likely to affect the baby's survival? SELECT ONE ANSWER ONLY A. Acute renal failure B. Lung hypoplasia C. Necrotising enterocolitis D. Peritonitis E. Surfactant deficient lung disease

Qu 31 An infant was born at 26-weeks gestation and ventilated for 4 weeks. He suffered from necrotising enterocolitis but recovered and is established on enteral feeds. He is now 6 weeks old. Which screening test is the most important to perform before his discharge home? SELECT ONE ANSWER ONLY A. Eye examination B. Guthrie test (PKU screen) C. Metabolic bone screen D. Oto-acoustic emission hearing test E. Serum TSH concentration

Qu 32 A 7-year-old boy presents with a 4-day history of bloody diarrhoea. He is not clinically dehydrated but looks pale and his urine output is poor. His blood pressure is 150/100mmHg. He has a nose bleed soon after admission. Initial blood results: haemoglobin 6.2 g/dl white cell count platelet count Which test should be done next? SELECT ONE ANSWER ONLY A. Abdominal ultrasound scan B. Bone marrow aspirate C. Coagulation screen D. Serum creatinine E. Stool sample for culture 15.7 x 10 /l
9

30 x 10 /l

Qu 33 A 12-month-old boy is brought in to the Emergency department in the early evening. He is unconscious (Glasgow coma scale level 6) and is found to have marks on his abdomen which look like bruises. His parents are both intoxicated and cannot give a reasonable explanation. Following stabilisation, which is the most urgent next step in management? SELECT ONE ANSWER ONLY A. CT brain scan to look for a subdural haematoma B. Blood count, plasma urea and electrolytes for suspected haemolytic-uraemic syndrome C. Intravenous cefotaxime to cover suspected meningococcaemia D. Skeletal survey for suspected non-accidental injury E. Urine and blood screen for toxicology

Qu 34 A mother brings her 6-year-old son to her General Practitioner requesting a referral for tonsillectomy. What is the most reliable indication for tonsillectomy? SELECT ONE ANSWER ONLY A. 6 attacks of pyogenic tonsillitis in the last 12 months B. Persistent mouth breathing C. Poor appetite in the last year D. Tonsillectomy in his elder brother when aged 12 years E. Tonsils meeting in the midline

Qu 35 A 12-year-old girl is being investigated for dyspnoea. Lung function tests: FVC FEV1 Peak expiratory flow

49% predicted 48% predicted 85% predicted

What is the best interpretation of the findings? SELECT ONE ANSWER ONLY A. Bronchial hyper-reactivity B. Hyperinflation C. Impaired gas exchange D. Restrictive lung disease E. Ventilation-perfusion mismatch

Qu 36 A 7-year-old boy is causing concern at school. His teacher has noticed that sometimes he concentrates well in class doing work as well or better than other children but at other times he is silly, will not concentrate and achieves little. He seems to be worse after eating chocolate. He was born by normal vaginal delivery and had been admitted to hospital once as an infant with screaming and was diagnosed as having colic. In his pre-school years he shouted and screamed when playing with other children but was healthy otherwise, passing all his preschool screening tests. After his sister was born his parents noticed that in comparison to her he was rather clumsy. Normally a loving child, he becomes extremely annoyed if his carefully arranged cars are moved. He is very keen on numbers, working out complicated arithmetical problems by his own methods. He is also very skilled with his hands. What is the best explanation of his difficulties? SELECT ONE ANSWER ONLY A. Attention deficit hyperactivity disorder B. Autistic spectrum disorder C. Exceptionally intelligent child who is bored D. Food intolerance E. Jealous of younger sibling

Qu 37 After an uneventful pregnancy and labour, a baby is born at term. He is noticed to be tachypnoeic and pale immediately after delivery. His haemoglobin is 7g/dl. What is the most appropriate investigation to elucidate the underlying cause? ANSWER SELECT ONE ANSWER ONLY A. Activated partial thromboplastin time B. Direct Coombs test C. Maternal Kleihauer test for fetal haemoglobin D. Plasma prothrombin time E. Reticulocyte count

Qu 38 A 4-year-old boy presents with difficulty in walking and "painful" legs. Blood haemoglobin 13.3 g/dl 9 white cell count 5 x10 9 platelet count 250 x10 ESR 2mm urea sodium potassium bicarbonate pH creatinine calcium phosphate alkaline phosphatase amino acids parathyroid hormone 2.0 mol/l 138 mmol/l 4.0 mmol/l 22 mmol/l 7.38 50 mol/l 2.0 mmol/l 0.8 1,000 U/l normal 16.4 pmol/l (4-8)

Which further investigation is the most likely to provide the diagnosis? SELECT ONE ANSWER ONLY A. Auto-antibody screen B. Liver function tests C. Plasma calcitonin levels D. Thyroid function tests E. X-ray of wrist

Qu 39 A 5-year-old boy presents with pubic hair development. On examination he is tall with th height on the 90 centile. He has scanty pubic hair development. His testes and penis are enlarged. His blood pressure is 105/60 mmHg and he has no palpable abdominal masses. His bone age is 9 years. What is the most likely diagnosis? SELECT ONE ANSWER ONLY A. Adrenal tumour B. Congenital adrenal hyperplasia C. Intracranial tumour D. Premature adrenarche E. Testicular tumour

Question 40 A baby boy was born at term weighing 3.1kg. There had been no abnormalities detected during full antenatal surveillance. He bottle-feeds well but develops progressive vomiting and abdominal distension. On day 6 the vomitus is bile-stained. What is the most likely diagnosis? SELECT ONE ANSWER ONLY A. Cows milk allergy B. Duodenal atresia C. Galactosaemia D. Gastro-oesophageal reflux E. Hirschprungs disease

Qu 41 A male infant is born at term weighing 1.3kg. What is the most likely cause for this? SELECT ONE ANSWER ONLY A. Chromosome abnormality B. Maternal cocaine abuse C. Maternal hypertension D. Maternal malnutrition E. Maternal smoking

Qu 42 A 14-year-old girl is brought by ambulance with no parents. She is unconscious, Glasgow Coma Scale of 9. Her clothing is dishevelled and she has bruising on her limbs. She has no focal neurological signs. What is the most likely cause of the clinical picture? SELECT ONE ANSWER ONLY A. Head injury B. Intentional overdose C. Intoxication D. Intracranial bleed E. Meningococcal meningitis

Qu 43 A 2-year-old girl presents with right sided cervical lymphadenopathy, right sided Horners syndrome and weight loss. Blood pressure 148/103 Chest X-ray mediastinal mass. Which investigation is most likely to give a diagnosis? SELECT ONE ANSWER ONLY A. Abdominal USS B. CT scan of the chest C. FBC and film D. Serum AFP and HCG E. Urinary VMA and HVA

Qu 44 An 8-year-old boy is referred because of joint pains following exercise. There had been concerns about his development initially and he did not walk until the age of 17 months. He is doing well at school since the prescription of corrective lenses. His single-parent mother and his sister are of average height. th Height 97 centile th Weight 50 centile. There is no joint swelling but it is noticed that he can extend his knees and elbows beyond 180. He has a grade 2/6 early-diastolic murmur heard best at the left upper sternal edge. A. Benign congenital hypotonia B. Ehlers Danlos syndrome C. Homocystinuria D. Marfans syndrome E. Osteogenesis imperfecta

Qu 45 A 14-year-old girl has a history of deliberate self-harm and recent weight loss. She has been vomiting overnight and is drowsy. Her breathing is laboured. Her mother thinks she took some tablets Blood sodium 126 mmol/l potassium 2.7 mmol/l chloride 100 mmol/l Blood gas pH pO2 pCO2 HCO3

7.5 8.2 kPa 4.0 kPa 28mmol/l

What is the most likely diagnosis? SELECT ONE ANSWER ONLY A. Ingestion of aspirin B. Ingestion of paracetamol C. Laxative abuse D. Type I diabetes mellitus E. Urinary tract infection

Qu 46 An 8-month-old boy is brought to the emergency department by his parents with a 12 hour history of vomiting. They say he fell from a chair in the kitchen 2 days ago and banged his head. He cried but was quickly pacified. You examine the child thoroughly and arrange investigations. Which finding is most likely to support a diagnosis of non-accidental injury ? SELECT ONE ANSWER ONLY A. A greenstick fracture of the left tibia B. A healed scald on the back of his hand C. A large purple bruise on the left side of the forehead D. A unilateral parietal fracture with overlying soft-tissue swelling E. Retinal haemorrhages

Qu 47 A 5-year-old girl has had recurrent blackouts for the last 6 months. They last less than a minute with rapid recovery. They occur without warning during the day, when she is active. During the turns some twitching of her hands and feet is seen. The girl is clinically and developmentally normal Her mother had similar episodes until adolescence. A maternal uncle died suddenly when a teenager. Routien EEG and ECG are normal, as are fasting blood glucose and calcium levels. What is the most likely diagnosis? SELECT ONE ANSWER ONLY A. Breath-holding attacks B. Epilepsy C. Long Q-T syndrome D. Reflex anoxic seizures E. Sick sinus syndrome

Qu 48 A 13-year-old boy with asthma is brought in from a discotheque to the emergency department with a sudden onset of breathlessness and wheeze. He looks agitated and tachypnoeic. Peak flow is 100 L/min and his heart rate is 130/min. Lung auscultation reveals equal but diminished breath sounds and bilateral wheeze. The ambulance crew administered oxygen by mask and nebulized salbutamol 20 minutes ago with little relief to his symptoms. What would be the most appropriate immediate step in the management of this child? SELECT ONE ANSWER ONLY A. Administer intravenous aminophylline B. Administer intravenous salbutamol C. Administer oral prednisolone D. Obtain a chest X-ray E. Repeat nebulized salbutamol

Qu 49 Cardiac catheterization of an 8-year-old boy gives the following results. Site Superior vena cava right atrium right ventricle pulmonary artery femoral artery What is the most likely diagnosis? SELECT ONE ANSWER ONLY A. Aortic stenosis B. Atrial septal defect C. Coarctation of the aorta D. Fallot's tetralogy E. Persistent arterial duct (PDA) pressure mm Hg 3 40/3 40/10 100/60 oxygen saturation (%) 75 73 74 85 98

Qu 50 A 6-year-old girl presents with a 24-hour history of abdominal pain, vomiting and poor urine output. Her temperature is 38.5 C, pulse 120/min, blood pressure 80/60mm Hg and capillary refill time 4 secs. Her abdomen is tender with guarding on rebound. After intravenous resuscitation she is taken immediately to theatres. She is found to have signs of peritonitis with ascites but a normal appendix. She is given intravenous antibiotics overnight. The next day she has developed facial swelling and has still not passes urine. Blood haemoglobin 9.6 g/dl 9 white cell count 18.3 x 10 /l sodium potassium bicarbonate urea creatinine albumin AST ALT alkaline phosphatase 137 mmol/l 4.9 mmol/l 18 mmol/l 17.9 mmol/l 79 mol/l 21 g/l 40 IU/l 38 IU/l 212 IU/l

What pathogen is most likely to be identified in the ascitic fluid? SELECT ONE ANSWER ONLY A. Escherichia coli B. Mycobacterium tuberculosis C. Streptococcus faecaelis D. Streptococcus pneumonia E. Yersinia enterocolitica

Qu 51 A 14-year- old boy presented 2 months ago with pain and swelling of his left knee and right elbow. The sole of his right foot is tender in the mornings. He had been well previously. His mother and brother have eczema Blood: haemoglobin 11.5 g/dl 9 platelets 362 x 10 /l 9 white cell count 9.8 x 10 /l 9 lymphocyte count 2.1 x 10 /l ESR 32 mm/hr He was commenced on oral Ibuprofen and referred to a physiotherapist. His joint pain improved but he now has abdominal pain, which is worse after meals, and diarrhoea. He also has lost some weight. In the past he has had no health problems. What is the most likely diagnosis? SELECT ONE ANSWER ONLY A. Crohns disease B. Enthesitis-related arthritis C. Hypermobility D. Systemic lupus erythematosis E. Systemic onset juvenile idiopathic arthritis

Qu 52 A baby is born at term with meconium staining. He cries immediately and is vigorous. What is the most appropriate management? SELECT ONE ANSWER ONLY A. Endotracheal intubation, suction and ventilation B. Endotracheal suction, antibiotics and observation C. Endotracheal suction and observation D. Oropharyngeal suction and observation E. Observation only

Qu 53 A baby boy was born at 38 weeks gestation. Labour had been induced because of concerns about reduced fetal movements. He was resuscitated with bag and mask. He was admitted to the neonatal unit with hypotonia when he was noted to be hypotonic. Now at 2 months of age he is alert and smiling but he feeds slowly and is very floppy. What is the most likely diagnosis? SELECT ONE ANSWER ONLY A. Cerebral palsy B. Congenital muscular dystropy C. Dystrophia myotonica D. Prader Willi syndrome E. Spinal muscular atrophy

Qu 54 A 14-year-old girl presents with a 3-month history of anorexia, pallor, and tiredness. th Her blood pressure is 140/95 mmHg. Her height and weight are both on the 50 centile. Blood haemoglobin 6.0 g/dl urea 35 mmol/l creatinine 800 mol/l phosphate 3 mmol/l (normal 0.8-1.8) pH 7.25 Urine testing blood protein Renal ultrasound

moderate ++++ small kidneys with increased echogenicity.

What is the most likely diagnosis? SELECT ONE ANSWER ONLY A. Chronic glomerulonephritis B. Haemolytic-uraemic syndrome C. Juvenile nephronophthisis D. Malignant nephrosclerosis E. Reflux nephropathy

Question 55 A 15-year-old boy has severe acne with whiteheads on his face and inflamed lesions on his back and chest. He has tried various treatments recommended by the local pharmacy as well as benzyl peroxide gel plus oral tetracycline. He is very distressed and is refusing to go to school or to play sports. What is the best management? SELECT ONE ANSWER ONLY A. Benzyl peroxide gel plus topical retinoid plus oral tetracycline B. Hormonal treatment C. Oral isotretinoin D. Referral to Child and Adolescent Mental Health E. Use a comedone extractor on the lesions

Qu 56 A pregnant woman is thyrotoxic. Which of the following is most likely to result in neonatal hyperthyroidism by maternal-fetal placental transfer? SELECT ONE ANSWER ONLY A. Iodine B. Thyroid stimulating hormone C. Thyroid stimulating immunoglobulin D. Thyroxine E. Tri-iodothyronine

Qu 57 A 2-year-old girl presents to the Emergency department in the early evening, fitting. Her mother said that she was well when she ate her evening meal. She is afebrile. The blood glucose is 0.7mmol/l. She has a past history of unexplained apnoeic attacks. Her mother, who has psoriasis, is a nurse. Her father, who has insulin dependent diabetes, is a lawyer. Her hypoglycaemia is treated, she is admitted and remains well. Capillary blood glucose monitoring for 24 hours is normal. What is the best management option at this stage? SELECT ONE ANSWER ONLY A. Child protection strategy meeting B. Emergency protection order C. Pituitary function tests D. Prolonged glucose tolerance test E. Urine metabolic screen

Qu 58 A 12-year-old boy, recently emigrated from the Indian subcontinent, has 17mm induration on Mantoux testing. He is asymptomatic and clinical examination is normal. A BCG scar is found. He has a normal chest X-ray. There is no history of close family contact with tuberculosis. What is the most appropriate management strategy now? SELECT ONE ANSWER ONLY A. Give chemoprophylaxis with isoniazid and rifampicin for 3 months B. Perform a laryngeal swab for acid-fast bacilli stain and culture C. Reassure and discharge from the clinic D. Repeat the chest X-ray in 4 months E. Treat for clinical tuberculosis for 6 months, including 2 months of triple therapy with isoniazid, ethambutol and rifampicin

Question 59 A 2 year old boy whose mother has chronic hepatitis B infection has the following serology tests results: Hep B s antigen Hep B s antibody Hep B e antigen Hep B e antibody Hep B c antibody HBV DNA positive negative negative positive positive negative

What is the most accurate assessment of his current hepatitis B status? SELECT ONE ANSWER ONLY A. Acute hepatitis B infection B. Chronic hepatitis B carrier (high infectivity) C. Chronic hepatitis B carrier (low infectivity) D. Post-hepatitis B immunisation E. Resolved hepatitis B infection

Question 60 A 6-year-old girl presents with short stature. She was born at term with a birth weight of 2.8 Kg. She was well in the newborn period. She has mild learning difficulties. th Height 0.4 centile th Weight 9 centile th Mid-parental height 50 centile Which investigation is most likely to lead to a diagnosis? SELECT ONE ANSWER ONLY A. Glucagon stimulation test B. Insulin-like growth factor 1 (IGF-1) C. Karyotype D. Skeletal survey E. Thyroid function tests

Qu 61 An 18-month-old girl is brought to the clinic because she is not yet walking. She was a home delivery at home weighing 3.2 Kg and there were no neonatal concerns. She rolled at 5 months and sat at 7 months. She is able to pull to stand but not yet cruising. Fine motor, vision, speech and language development are age-appropriate. Which investigation is most likely to lead to a diagnosis? SELECT ONE ANSWER ONLY A. Chromosomes B. Creatine kinase C. Full blood count and iron studies D. Thyroid function tests E. X-ray of hips

Qu 62 A 5-year old boy is brought by his mother because he will not go to sleep without a dummy (pacifier) and he will not settle until he is given a beaker of milk. He has also started to wet his bed. He used to sleep well. His parents think the problem started when he started to stay for lunch at school 6 months ago. After falling asleep he wakes up very distressed. He tells his mother that he sees things and is frightened. What is the most likely explanation? SELECT ONE ANSWER ONLY A. Food intolerance B. Night terrors C. Nightmares D. Nocturnal epilepsy E. Urinary tract infection

Qu 63 A girl was born at term weighing 3.4kg. Her Apgar scores were 8 at 1 minute and 10 at 5 minutes. She smiled at 9 weeks. At the age of 3 months her parents report that she does not follow them around the room with her eyes. Ophthalmoscopic examination and developmental assessment in the clinic are normal. What is the most appropriate next action? SELECT ONE ANSWER ONLY A. Arrange an EEG B. Refer for a full neuro-developmental assessment C. Refer to a paediatric ophthalmologist D. Refer to an orthoptist E. Review in 3 months

Qu 64 A baby boy weighing 4 kg has just been circumcised. Shortly after returning to the ward he is found to be very unwell having bled significantly from the wound. You are asked to see him urgently and find him to have a pulse rate of 160/minute with low volume, capillary refill time of 3 seconds and a respiratory rate of 70/min. You insert an intravenous cannula. Which of the following would you give first? SELECT ONE ANSWER ONLY A. 0.9% sodium chloride B. 4.5% human albumin solution C. Fresh frozen plasma D. Group O Rh negative blood E. IV vitamin K

Qu 65 A 1-month-old boy is brought to the Emergency department. His mother says he has been breathless and is feeding poorly and this has become progressively worse over the past 10 days. Examination: Colour pink with O2 saturation of 94% Respiratory rate 60/min Heart rate 170/min Liver 3 cm below the costal margin Femoral pulses equal in volume to the right brachial Parasternal heave marked Heart sounds normal Murmur moderately loud and long systolic at the lower left sternal edge. Investigations: Chest X-ray ECG

Cardiothoracic (CT) ratio of 0.6 with plethoric lung fields. Moderate left ventricular hypertrophy.

After admission to hospital, which is the most appropriate therapy? SELECT ONE ANSWER ONLY A. Give 3 doses of oral indometacin B. Restrict milk feeds and other fluids to 100 ml/kg/day C. Start oral captopril D. Start oral digoxin therapy E. Start oral diuretic therapy

Qu 66 A 2 year-old boy had been born at 29 weeks gestation weighing 0.96kg. He can now understand simple instructions and speak using 3-word combinations. He can sit unsupported, can feed himself and can stand but he cannot walk unaided. What is the most likely diagnosis? SELECT ONE ANSWER ONLY A. Athetoid cerebral palsy B. Developmental co-ordination disorder C. Global developmental delay D. Spastic diplegia E. Spastic quadriplegia

Qu 67 A 13-year-old boy has an afebrile generalised seizure which lasts 3 to 5 minutes before full recovery. His first seizure occurred at the age of 7 years and he has had 6 episodes at roughly yearly intervals. All the fits were similar in pattern and occurred at home. EEG at presentation showed generalised spike and wave activity. He is generally very well and active and is making good progress at school. Both mother and patient do not want him to have any antiepilepsy drug treatment. What is the best management option? SELECT ONE ANSWER ONLY A. Agree not to treat with antiepilepsy drugs unless fit frequency increases significantly B. Persuade mother and child to accept antiepilepsy drug therapy on account of the risk of SUDEP (sudden unexpected death in epilepsy) C. Postpone the decision until he has had an MRI head scan D. Postpone the decision until he has had another EEG E. Request social service to convene a case conference

Qu 68 A baby girl was delivered at full term in good condition following an uneventful pregnancy and labour. Her mother has long-term epilepsy which is well controlled with sodium valporate. The infant received a 2 mg dose of vitamin K orally shortly after birth. Mother and baby were discharged home on the second day of life. She presents at 4 days of age with bleeding from the umbilical stump. She is otherwise well and breast feeding satisfactorily. What is the most likely diagnosis? SELECT ONE ANSWER ONLY A B C D E Alloimmune thrombocytopenia Disseminated intravascular coagulation Haemophilia A Umbilical granuloma Vitamin K deficiency bleeding

Qu 69 A 6-month-old girl with fever, vomiting and diarrhoea is admitted to the paediatric ward for observation. She is not dehydrated and is tolerating oral fluids. The family have been given a leaflet about oral rehydration therapy. Meanwhile a nurse notices a bruise on her inner thigh. On asking the parents about the marks you notice that the mother has facial bruising and that the father smells of alcohol. The father becomes verbally abusive, the mother picks up the baby and while you are talking to them they announce their intention to leave the hospital and start walking towards the ward exit. You ask the family politely to stay but they walk past you. What is the best action to take next? SELECT ONE ANSWER ONLY A. Allow her to go home on oral rehydration therapy but telephone the social worker indicating your concerns B. Ask a nurse to telephone the Police C. Call for the hospital Security Officer (has no right to restrain parents) D. Check the Child Protection Register E. Say to the parents that you are very worried about a blood disorder and ask them to agree to a blood sample being taken

Qu 70 Shortly before giving birth at full term a mother is diagnosed as having active tuberculosis. She has 2 other children, aged 2 and 4 years, who are reported to be well. What is the most important action to advise? SELECT ONE ANSWER ONLY A. The baby should be fed with the mothers expressed breast milk B. The baby should be treated with isoniazid C. The baby should have a chest X-ray before discharge home D. The baby should have BCG immunisation at birth E. The mother should be offered HIV testing before discharge

Qu 71 A 3-month-old girl presents with a 1-day history of irritability, vomiting and decreased feeding. She has a high fever with temperatures reaching 39.5C. She is lethargic and the capillary refill is 3 seconds. She is not tachypnoeic and no focus of infection can be identified on examination. What is the most likely diagnosis? SELECT ONE ANSWER ONLY A. Bacterial pneumonia B. Gastroenteritis C. Otitis media D. Upper respiratory tract infection E. Urinary tract infection

Qu 72 A 9-year-old boy presents with a history of painless haematuria confirmed on microscopy. This has occurred on 4 occasions over the past 6 months, each time he has had febrile illnesses. There has been no dysuria and he is otherwise well. There are no abnormalities on examination; blood pressure is 115/65 mmHg What is the most likely diagnosis? SELECT ONE ANSWER ONLY A. Alports Syndrome B. Henoch-Schnlein nephritis C. IgA nephropathy D. Minimal change nephropathy E. Post-streptococcal glomerulonephritis

Qu 73 A 3-year-old girl presents with a fluctuant discoloured lump in the anterior triangle of the neck. It had been present for 2 months in spite of 2 courses of amoxicillin. Results of needle aspiration were unhelpful and culture was negative. On follow-up she developed a fistula which has not healed despite a 6-week course of clarithromycin and ciprofloxacin. She was an otherwise healthy girl, born in London, and was fully immunised. What is the most likely diagnosis? SELECT ONE ANSWER ONLY A. Atypical mycobacterial adenitis B. Cat-scratch disease C. Hodgkins lymphoma D. Infected branchial cyst E. Tuberculous lymphadenitis

Qu 74 A 5-year-old girl is brought to the Accident and Emergency Department with rash of th sudden onset, hyperactivity and poor sleeping. Her height and weight are on the 75 centiles. Her parents say that she is taking medicine for epilepsy but they cannot remember the name of the drug. They had recently renewed the prescription and the pharmacist had wondered whether this was the correct dose. Which drug is the most likely to have caused this problem? SELECT ONE ANSWER ONLY A. Carbamazepine B. Ethosuximide C. Lamotrigine D. Phenytoin E. Sodium valproate

Qu 75 A previously healthy 13-year-old boy presents with a 4-week history of excessive tiredness. He has been unable to take part in football, which he enjoyed very much previously. Other symptoms include occasional brief episodes of dizziness on waking up in the mornings, recent weight loss and nocturia. He has difficulty in concentration at school and performed poorly in the recent examinations. His height and weight are both on the 10 centile. He is afebrile with a heart rate of 140/min and blood pressure of 90/50 mm Hg. Blood haemoglobin 14.9 g/dl 9 total white cell count 13.1 x 10 /l 9 platelets 356 x 10 /l ESR 10 mm/hr C-reactive protein (CRP) <5.0 mg/L sodium potassium urea creatinine total protein albumin globulin total bilirubin alkaline phosphatase alanine aminotransferase (ALT) gamma-glutamyl transpeptidase (GGT) Urine osmolality dipstix 126 mmol/l 5.0 mmol/l 6.6 mmol/l 51 mol/l 73 g/l 41 g/l 32 g/l 4.1 mol/l 189 U/l 20 U/l (normal 5-45) 8 U/l (normal 5-24)
th

826 mosm/kg negative for glucose

Which further investigation is most likely to yield a diagnosis? SELECT ONE ANSWER ONLY A. Epstein Barr virus (EBV) serology B. Glucose tolerance test C. Growth hormone stimulation test D. Short synacthen test E. Thyroid function tests

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