Pediatrics MCQ

Pediatrics 1) Peeling of the palms of the hand and the soles of the feet is a characteristic feature of: A Chickenpox.

(False)

B Scarlet fever.

(True)

C Kawasaki disease.

(True)

D Tinea infection.

(True)

E Papular urticaria.

(False)

Comments: Both chicken pox and popular urticaria are associated with encrusted vesicles. Kawasaki disease is associated with high temperature, mucosal inflammation and desquamation.

2) A term newborn infant is noticed to be hypotonic. The following are possible causes:

A Turner's syndrome.

(False)

B Kugelberg-Welander disease.

(False)

C Cephalhaematoma.

(False)

D Prader-Willi syndrome.

(True)

E Galactosaemia.

(True)

Comments: Causes of neonatal hypotonia can be classified anatomically: Systemic: sepsis, trisomies, metabolic disease, e.g. galactosaemia, drugs, etc Brain: hypoxic ischaemic encephalopathy, intracranial haemorrhage, malformations Spinal cord: birth trauma, spina bifida, tumour Anterior horn cell: spinal muscular atrophy Neuromuscular junction: neonatal myasthenia gravis Muscle: congenital myopathies, benign congenital hypotonia.

3) Respiration is stimulated by the following: A Hypercarbia.

(True)

B Hypoxia.

(True)

C Acidosis.

(True)

D Increased barometric pressure.

(False)

E Carbon monoxide inhalation.

(False)

Comments: Respiration is stimulated primarily by medullary chemoreceptors which respond to

Pediatrics

1

hypercarbia. The presence of acidosis exaggerates this response. Oxygen only stimulates respiration if levels are very low, e.g. at high altitude (low barometric pressure). This also becomes more important in chronic lung disease when carbon dioxide levels are chronically raised. From Hannam et al. MRCP (Paediatrics) Part 1 MCQs. page 67 © WB Saunders. Reproduced with permission.

4) Which of the following features supports a diagnosis of carditis in a child suspected of having rheumatic fever?

A Pan-systolic murmur in the mitral area.

(True)

B Elevated sleeping pulse.

(False)

C Sinus arrhythmia.

(False)

D Ejection systolic murmur at the left sternal border.

(False)

E Pericardial rub.

(True)

Comments: Pan systolic murmur indicates mitral incompetence. Elevated sleeping pulse though a feature, is not diagnostic. The classical valve lesions are aortic and mitral incompetence. Pericarditis indicates cardiac involvement and almost always occurs with valvulitis.

5) Early cardiac failure during the neonatal period is often associated with:

A Peripheral oedema

(False)

B Failure to thrive

(True)

C Feeding difficulties

(True)

D Episodes of apnoea

(True)

E A raised pulse rate but normal respiratory rate

(False)

Comments: Oedema occurs uncommonly in neonates. Failure to thrive, feeding difficulties and apnoeic episodes are common. Tachycardia and tachypnoea with respiratory rate of above 60 per minute are common. 6) A normal 4 year old would be expected to be able to: A Catch a ball.

(True)

B Draw a circle.

(True)

C Count to 10.

(True)

D Tell you his/her name and address.

(True)

Pediatrics

2

E Get dressed alone.

(True)

Comments: It is essential to have some knowledge of each of the four areas of child development (i.e. gross motor, fine motor and vision, hearing and speech and social behaviour) for several age groups. We suggest learning two to three normal milestones for each area of development for the following ages: 6 weeks, 6-8 months, 12 months, 2.5 years, 3 years, 4 years and 5 years. From Hannam et al. MRCP (Paediatrics) Part 1 MCQs. page 169 © WB Saunders. Reproduced with permission.

7) The following may cause deafness in children A meningococcal septicaemia

(True)

B maternal rubella

(True)

C congenital syphilis

(True)

D hereditary nephritis

(True)

E Pendred’s syndrome

(True)

Comments: 1.Deafness is an important complication of meningitis in children 2. Deafness occurs in up to 80% of children affected by congenital rubella 3. Nerve deafness can be a late manifestation of congenital syphilis 4. Alport’s syndrome is the combination of nerve deafness and hereditary nephritis 5.Pendred’s syndrome – nerve deafness and hypothyroidism – due to an inborn defect of organification of trapped iodine

8) A 6-week-old male infant is admitted to hospital with 10-15% dehydration. The serum sodium is 160 mmol/l and the serum potassium is 3.3 mmol/l:

A Congenital adrenal hyperplasia is a likely diagnosis.

(False)

B Loss of skin turgor is likely to be present.

(False)

C The dehydration should be corrected over the next 2-4 hours.

(False)

D Bloody diarrhoea suggests a bacterial cause.

(True)

E Initial fluid replacement should be with 0.45% saline.

(True)

Comments: The most likely cause of hypernatraemic dehydration in infants and children is infective diarrhoea. This is likely to be bacterial if the stool contains blood. The skin may have a doughy consistency in hypernatraemic dehydration. Rapid correction of the increased serum osmolarity results in intracellular fluid accumulation, which may lead to cerebral oedema. This can be avoided by slowly correcting the fluid depletion (over 24-48 hours) and ensuring that the serum sodium is lowered at a rate of no greater than 10 mmol/l/24 hours.

Pediatrics

3

9) The following are associated with neonatal hypoglycaemia:

A Down's syndrome.

(False)

B Reye syndrome.

(False)

C nesidioblastosis.

(True)

D Galactosaemia.

(True)

E Polycythaemia.

(True)

Comments: Causes of neonatal hypoglycaemia can be classified as follows: 1. Hyperinsulinism (maternal diabetes - islet cell hyperplasia - Beckwith-Wiedemann syndrome Nesidioblastosis - islet cell adenoma). 2. Decreased production (prematurity - ntrauterine growth retardation - glycogen storage disease glactosaemia). 3. Increased utilization (hypothermia – polycythaemia – asphyxia – sepsis). 4. Inborn errors (maple syrup urine disease (MSUD), - proprionic acidaemia, tyrosinaemia). 5. Endocrine causes (panhypopituitarism, - adrenal insufficiency). 6. Miscellaneous (maternal drugs (beta agonists/blockers, thiazides), - post-exchange transfusion).

10) The following statements regarding an atrioventricular septal defect (AVSD) are correct:

A Most defects occur in children with Down's syndrome.

(False)

B Congestive heart failure usually occurs by 4-8 weeks of age.

(True)

C Without surgical treatment most infants will die between the ages of 2 and 3 years.

(True)

D An ECG rarely shows right ventricular hypertrophy (RVH) or right bundle branch block (RBBB).

(False)

E A loud 3-4/6 pan systolic murmur is audible along the left sternal edge (LSE).

(True)

Comments: AVSDs account for about 2% of all congenital heart disease. Children with Down's syndrome only account for about 30% of all AVSDs. Heart failure occurs early and requires aggressive medical treatment. Recurrent chest infections are common. Surgical intervention is essential for long-term survival. An ECG shows left anterior hemi block (superior QRS axis), a QRS axis between -40 degrees and -150 degrees. RVH or RBBB is present in all patients and left ventricular hypertrophy may occur. First-degree atrioventricular block with a prolonged PR interval is also common.

Pediatrics

4

11) Which of the following statements regarding Napkin rashes are correct?

A Always caused by ammonia.

(False)

B Less common in babies fed a soy protein milk.

(False)

C Often successfully treated by exposure to air.

(True)

D Evidence of early infantile eczema.

(False)

E More common in boys.

(False)

Comments: Causes of napkin rash include contact dermatitis, which may produce ammonia and this may burn the skin. Infection with bacteria and candida yeasts may cause nappy rash, as can psoriasis and atopic dermatitis affecting the nappy area. Nappy rash is not an indicator of infantile eczema, and it is not more common in boys, nor is it less common in soy fed infants. Treatment is best achieved by prevention by frequent (disposable absorbent) nappy changing, and fluid feeding early in the day to lessen night time urination. Anti-fungal and Lotrimin lotions may also be useful.

12) Cyanosis in the first few days of life is likely with: A Transposition of the great vessels

(True)

B Tetralogy of Fallot

(False)

C Tricuspid artresia

(True)

D Atrial septal defect

(False)

E Coarctation of the aorta

(False)

Comments: Tricuspid atresia and Transposition of the great arteries are both cyanotic congenital heart diseases and present in the immediate newborn period. Tetralogy of Fallot can manifest at any time and sometimes in early childhood. ASD is a left to right shunt and presents a few months after birth. Cyanosis develops if there is Eisenmenger’s syndrome. Coarctation is a narrowing of the aortic arch.

13) The following statements relating to the development of the human brain are correct:

A The primitive brain vesicle appears at the end of the 4th week.

(True)

B The cerebral hemispheres result from evagination of the prosencephalon.

(True)

C The diencephalon is adjacent to the IIIrd ventricle.

(True)

D The cerebellum starts as a projection into the IVth ventricle.

(True)

Pediatrics

5

E The nervous system develops from the ventral ectoderm at about the 16th day.

(False)

Comments: Development of the brain begins with the appearance of the brain vesicles in the early part of the embryonic period. Evagination of the prosencephalon gives rise to the cerebral hemispheres. The primitive hemispheres and lamina terminalis constitute the telencephalon (endbrain). The diencephalon (epithalamus, thalamus and hypothalamic apparatus) is adjacent to the IIIrd ventricle. The cerebellum develops from a projection into the IVth ventricle. The nervous system dedelops from Dorsal ectoderm at the 16th day. The neural tube begins to fuse at the end of the 3rd week. Rostral closure occurs on the 24th and caudal closure on the 26th day.

14) A small ventricular septal defect is associated with: A A pansystolic murmur heard best at the end of the sternal edge

(True)

B A high risk of infective endocarditis

(True)

C Fixed splitting of the second sound

(False)

D Cyanosis

(False)

E A mid-diastolic murmur at the apex

(False)

Comments: A small VSD is classically associated with the holosysolic murmur at the left sternal edge; The risk of infective endocarditis is greater in haemodynamically insignificant defects. The second heart sound may be obscured by the murmur, but is normally split. Cyanosis occurs if there is Eisenmenger’s syndrome. A mid diastolic murmur at the apex is associated with a moderate sized shunt and increased flow through the left atrium.

15) Regarding achondroplasia: A Usually a hereditary condition

(True)

B Have proportionate trunk and limb lengths

(False)

C The individual usually has decreased mental development

(False)

D Could lead to lumbar lordosis

(True)

E X-ray shows short dense bones with flaring of the ends

(True)

Comments: Achondroplasia is usually a hereditary condition but may also occur sporadically. It particularly affects the pre-bone cartilage of long bones at birth resulting in Dwarfism. The affected individuals have a normal trunk length but short stumpy limbs all of the same length. They also have a large head with a saddle nose. Adult dwarfs have increasing lumbar lordosis, bow legs, and shortened proximal arms and legs. Intelligence level is usually normal. X-rays show short dense bones with flared ends with wide epiphysis.

Pediatrics

6

16) In children with Fallot’s tetralogy A Cyanosis is always present shortly after birth

(False)

B Heart failure is a common complication

(False)

C Auscultation reveals a systolic ejection murmur in the pulmonary area and a single second heart sound

(True)

D Squatting relieves cyanosis after exercise

(True)

E Functional anaemia may be present in spite of a haemoglobin concentration which is normal to age.

(True)

Comments: Cyanosis usually occurs 3-6 months after birth and may present in the first few years of life. Heart failure is not generally a feature of this condition. Systolic ejection murmur is present and the intensity is inversely proportional to the degree of Rt ventricular outflow obstruction. Pulmonic valve closure is not heard and hence single S2. Squatting is uniquely characteristic of a right to left shunt, and relieves cyanosis in exercise by decreasing systemic venous return. Prolonged cyanosis causes secondary polycythaemia which increases oxygen carrying capacity, therefore normal Hb concentration may cause symptomatic anaemia.

17) Galactosaemia A is an autosomal recessive condition

(True)

B cataract does not occur

(False)

C can cause jaundice

(True)

D can cause hypoglycaemia in the neonate

(True)

E can be improved by excluding milk from the diet

(True)

Comments: 2. cataracts do occur, usually bilaterally, and are reversible. 3. An important cause of prolonged jaundice in the neonate 4. A well recognized cause of hypoglycaemia 5. galactose is ingested as lactose in milk

18) The following conditions cause an increased anion gap:

A Diabetic ketoacidosis.

(True)

B Proximal renal tubular acidosis.

(False)

C Aspirin poisoning.

(True)

D Lactic acidosis.

(True)

E Acute renal failure.

(True)

Pediatrics

7

Comments: The anion gap is normally about 12 mmol/l. It is the difference between the cations (principally sodium and potassium) and the anions (principally consisting of chloride and bicarbonate ions). A rise in the gap shows the presence of an unmeasured cation or anion. In diabetic ketoacidosis this is beta-hydroxybutyrate and acetoacetate and in renal failure retained phosphate and sulphate. In proximal renal tubular acidosis the defect is one of bicarbonate loss and the anion gap remains unchanged.

18) A “strawberry” naevus is: A Usually present at birth.

(False)

B Best treated immediately it is recognized.

(False)

C Pulsatile.

(False)

D Often present at the nape of the neck.

(False)

E A capillary haemangioma.

(True)

Comments: A “strawberry” naevus is commonly present at birth but may not present till later on. Treatment is by laser or tattooing at a much later date. The majority occur in the regions supplied by the fifth cranial nerve. It is non pulsatile The other names are naevus flammeus and capillary haemangioma.

19) The following statements regarding classical phenylketonuria (PKU) are correct:

A Eczema is characteristically present in young children with the disease.

(True)

B Phenylalanine is not detectable in cord blood in newborn infants with PKU.

(True)

C Dietary restrictions are stopped after the age of 10 years.

(False)

D A pregnant woman with PKU will not put her baby at risk if she is on an unrestricted diet.

(False)

E Vomiting is an early presenting symptom of the disease.

(True)

Comments: Classical PKU is the result of a deficiency of phenylalanine hydroxylase. Eczema is often present in younger children, which disappears as the child grows older. Phenylalanine levels may rise to levels that are detectable by the Guthrie test as soon as 4 hours after birth, even before the ingestion of protein containing milk. It is recommended that a diet low in phenylalanine is continued for life. It has been shown that changes in white matter on stopping dietary restrictions are reversible on resumption of a low phenylalanine diet. It is essential that women with PKU should be on a diet low in phenylalanine in the preconception period to avoid damaging a normal fetus.

Pediatrics

8

20) Which of the following is/are recognized causes of alopecia in children:

A Alopecia areatea.

(True)

B Ectodermal dysplasia.

(True)

C Naevus flammeus.

(False)

D Ringworm.

(True)

E Trichotillomania.

(True)

Comments: Alopecia areata is autoimmune destruction of hair follicles. Ectodermal dysplasia is characterized by absent or deficient function of at least 2 derivatives of the ectoderm eg teeth and hair. Trichotillomania is an impulsive need to pull out hair from scalp and eyelashes. Ringworm is a fungal infection which results in hair loss. Naevus flammeus is another name given to a capillary haemangioma or port-wine stain.

21) The following infections are caused by gram-positive organisms: A Whooping cough.

(False)

B Typhoid.

(False)

C Diphtheria.

(True)

D Ophthalmia neonatorum.

(False)

E Tetanus.

(True)

Comments: Causative organisms are as follows: Whooping cough - Bordetella pertussis Typhoid Salmonella typhi Diphtheria - Corynebacterium diphtheriae Ophthalmia neonatorum Neisseria gonorrhoeae Tetanus - Clostridium tetani N.B: TTT of gram +ve organism’s need 10 day, gram –ve organisms need 14 days.

Gram +ve cocci Staph. aureus Strep. pyogenes Strep. pneumoniae Strep. viridans

Gram +ve bacilli Clostridium sp. Corynebacterium diphtheriae Listeria sp. Bacillus anthracis

Gram -ve cocci N. meningitidis N. gonorrhoeae

Gram -ve bacilli E. coli Salmonella sp. Shigella sp. Proteus sp. Klebsiella sp. Haemophilus sp. Bordetella pertussis Pseudomonas sp.

Pediatrics

9

22) A 5-year child presents with progressive weakness. The following make a diagnosis of Guillain-Barre syndrome more likely:

A Isolation of Campylobacter jejuni from the stool.

(True)

B leg pain

(True)

C dilated pupils

(False)

D The presence of over 50 lymphocytes/ml in the cerebrospinal fluid (CSF).

(False)

E Asymmetric weakness of the lower limbs.

(False)

Comments: Guillain-Barre syndrome (GBS) has been shown to be associated with C. jejuni infection. Pain is often a predominant feature of GBS and is the presenting symptom in up to 20% of cases. Children who present with pain often have significant emotional lability associated with vomiting and a headache. Pupillary abnormalities are not seen in GBS and when present suggest diptheria or botulism. The presence of over 50 lymphocytes/ml of CSF suggests polio or central nervous system lymphoma. In GBS the protein level in the CSF is typically 80-200 mg/dl. Paralysis of the lower limbs is usually symmetrical and is frequently associated with distal paresthesias and numbness but in poliomyelitis asymmetrical paralysis (in polio. No sensory involvement).

23) Parents of autistic children: A Commonly belong to higher social class.

(True)

B Have an increased incidence of schizophrenia.

(False)

C Should be warned about 1 in 4 risk of autism in future children.

(False)

D Can look forward to their child attending normal schooling.

(False)

E Commonly blame themselves unrealistically for their child’s behaviour.

(True)

Comments: A bias towards higher occupational class has been found in some studies. There is no increased prevalence of schizophrenia in the parents Between one third and two thirds of children are severely mentally retarded. The cause of autism is possibly related to complex genetic and environmental factors, specific risk factors for autism have not been identified.

Pediatrics

10

24) The following occur in childhood asthma. A cough as the predominant symptom

(True)

B stridor

(False)

C increased airways resistance

(True)

D decreased lung compliance

(False)

E decreased functional residual capacity

(False)

Comments: Persistent cough, especially at night, is often a presenting feature of childhood asthma. Stridor is a feature of upper airways obstruction and therefore does not occur in asthma. This leads to air trapping and increased Functional Residual Capacity (FRC). Compliance is normal.

25) The following viral infections are paired with their appropriate incubation periods:

A Measles - 10-12 days.

(True)

B Rubella - 7-14 days.

(False)

C Varicella zoster - 7-10days.

(False)

D Mumps - 14-24 days.

(True)

E Hepatitis B - 60-110 days.

(True)

Comments: The incubation period of rubella is 14-21 days. Varicella zoster virus has an incubation period of 13-17 days.

26) The following statements are correct: A Insulin is derived from C-peptide.

(False)

B Glucagon stimulates insulin release.

(True)

C C-peptide levels are increased in endogenous hyperinsulinaemia.

(True)

D Insulin decreases absorption of glucose from the gut.

(False)

E Catecholamines antagonize the actions of insulin.

(True)

Comments: Insulin is derived from proinsulin, which is cleaved to form insulin and C-peptide. C-peptide can be measured to distinguish between endogenous hyperinsulinaemia and exogenously administered insulin. Insulin inhibits glucagon release, whereas glucagon stimulates insulin release. Insulin has no effect on the absorption of glucose from the gut. Catecholamines inhibit the action of insulin.

Pediatrics

11

27) The following statements apply to a cephalhaematoma in the newborn.

A It usually manifests immediately after birth.

(False)

B The haematoma is formed beneath the periosteum.

(True)

C A common site is over the parietal bone.

(True)

D The treatment of choice is aspiration.

(False)

E Mother can be told it will disappear by the age of four weeks.

(False)

Comments: It usually manifests several hours after delivery. The haematoma occurs as a result of damage to capillaries beneath the periostium.They usually require no treatment, but may take up to 3 months to resolve. (beneath = under) 28) Regarding congenital dislocation of the hip: A The incidence is 2.5 to 20 per 1000 live births.

(True)

B The Ortolani-Barlow manoeuvre is highly sensitive and specific.

(False)

C It may be associated with polyhydraminos.

(False)

D The male: female ratio is 2:1.

(False)

E A family history is frequently found.

(False)

Comments: The incidence of unstable hips at birth is approximately 2%, of which 90% are dislocatable, and 10% dislocated. There is a 6:1 female to male ratio, left side > right side, and there may be a family history. It is commoner in first children, breech presentations (50% of infants with CDH), oligohydraminos, and may be associated with other deformities such as (talipes = club foot) • •

The Ortolani manoeuvre: detects a dislocated hip, as the hip is 'clunked' back into position. Barlow's test: detects a dislocatable hip by pressing posteriorly and internally rotating it, the hip can be dislocated.

The introduction of routine testing of the hips has not decreased the incidence of established dislocation. This may be because of poor examination technique, a hip that becomes unstable only when weight bearing begins, or an acquired dislocation with age. Late presentation of CDH may include asymmetry of skin folds, limited abduction of hips, shortening of the thigh, with knees at different level when the patient is supine and the legs are flexed (Galeazzi sign), and limping.

Pediatrics

12

29)The following are side-effects of sulphasalazine: A Reversible neutropenia.

(True)

B Acute pancreatitis.

(True)

C Methaemoglobinaemia.

(True)

D Permanent oligospermia.

(False)

E Folate deficiency.

(True)

Comments: Sulphasalazine is a chemical combination of sulpha-pyridine and 5-aminosalicylic acid (5-ASA) and is useful in the treatment of ulcerative colitis. 5-ASA is the active moiety and sulphapyridine acts as a carrier to the colonic site of action. Common side-effects are nausea, vomiting, epigastric pain, headache and rash, occasionally fever, anaemia, reversible neutropenia, folate deficiency and reversible oligospermia. Rarer side-effects include methaemoglobinaemia, pancreatitis, hepatitis, thrombocytopenia, agranulocytosis, aplastic anaemia and Stevens-Johnson syndrome.

30) Pulmonary hypertension: A Causes a diminished pulmonary second sound

(False)

B Causes left ventricular hypertrophy

(False)

C Commonly complicates large ventricular septal defects

(True)

D Results in a diminished main pulmonary artery shadow on chest x-ray

(False)

E Is a feature of the Eisenmenger’s syndrome

(True)

Comments: Pulmonary hypertension causes a loud P2 which may be palpable and causes right ventricular hypertrophy. Large ventricular defects are associated with increased pulmonary vascular resistance and clinical signs of pulmonary hypertension. The main pulmonary artery segment is prominent on the chest x-rays and the vascularity in the outer one third of the lung fields is reduced. Eisenmenger’s syndrome is characterized by pulmonary hypertension following reversal of a left to right shunt. 31) Vesicles are characteristic in A Exfoliative dermatitis

(False)

B Erythema nodosum

(False)

C Dermatitis herpetiformis

(True)

D porphyria cutanea tarda

(True)

E Steven's-Johnson syndrome

(True)

Pediatrics

13

Comments: Vesicles occur in erythema multiforme (Steven's Johnson), chicken pox, herpes zoster, Porphyria cutanea tarda, dermatitis herpetiformis and pemphigus / pemphigoid. Not erythema nodosum - raised tender nodules usually on the shins nor exfoliative dermatitis.

32) Regarding Vitamin K: A Modern formula feeds contain sufficient Vitamin K, but babies who are entirely or mainly breast fed should receive supplements.

(True)

B There is unequivocal evidence to suggest that intramuscular Vitamin K at birth increases the risk of childhood cancer.

(False)

C The ideal oral Vitamin K regimen is 3 doses given at birth, 7days and 28 days.

(True)

D Newborn infants have very low levels of Vitamin K which is needed for normal clotting.

(False)

E Deficiency most often presents at 3 months of age.

(True)

Comments: Deficiency of Vitamin K can lead to haemorrhage at a variety of sites. This may occur during the first few days (early haemorrhagic disease) or within the first 3 months of life (late haemorrhagic disease). The latter is much more serious because of the potential for intracranial bleeds, leaving 30% dead and 40% seriously handicapped. Modern formula feeds are supplemented with Vitamin K, but babies who are breast fed should definitely receive supplements. There is no definite link of intramuscular Vitamin K with childhood cancer, and the larger studies suggest that there is no link at all. Oral regimens of prophylaxis are likely to be suboptimal for compliance particularly from the third dose (in one study measured there was only 40%).

33) In children: A Moles are usually present at birth.

(False)

B Giant hairy moles may undergo malignant change.

(True)

C Malignant melanoma is very rare under the age of 10 years.

(True)

D All moles on the palms and soles should be excised prophylactically.

(False)

E Moles can appear at any time after birth.

(True)

Comments: Only 1% of moles are present at birth and termed congenital naevi. 3-10% of giant congenital moles (9cm on head and 6cm on the body) can give rise to melanoma. Small moles occurring on the palms and soles may be entirely benign and should be observed for signs of change. Moles can appear throughout life.

Pediatrics

14

34) Neuroblastoma A is an embryonic tumour

(True)

B usually has a familial incidence

(False)

C metastasises most commonly to the lungs

(False)

D is easily eradicated by radiotherapy

(False)

E is a secreting tumour

(True)

Comments: Neuroblastoma is a tumour derived from neural crest tissue and so, is properly regarded as an APUD tumour. The most common sites are: 50% adrenal medulla, 25% abdominal sympathetic ganglia, 20% chest, 5% pelvis, 5% neck. The tumour may secrete catecholamines hence urine VMA concentrations are elevated Spontaneous remission is well recognized in this condition. Extra abdominal sites generally have a better prognosis.

35) Hyponatraemia can occur in: A Congenital adrenal hyperplasia

(True)

B Gastroenteritis

(True)

C Diabetes insipidus

(False)

D Diabetes mellitus

(True)

E Severe pneumonia

(True)

Comments: Hyponatraemia occurs in congenital adrenal hyperplasia due to failure to synthesise cortisol and mineralocorticoid. Gastroenteritis may cause loss of sodium and water through and hence hyponatraemic dehydration. Diabetes insipidus causes hypernatraemia. Diabetes mellitus may cause osmotic diuresis and associated tubular sodium loss. Pneumonia may cause inappropriate ADH secretion and hyponatraemia results.

36) The following conditions may be detectable by growth monitoring: A Hyperthyroidism

(False)

B Hypothyroidism

(True)

C Pseudohypoparathyroidism

(False)

D XYY Syndrome

(False)

E Insulin dependent diabetes mellitus

(False)

Comments:

Pediatrics

15

Benefits of growth monitoring include: Early detection of conditions such as: • • • • • • • • •

Hypothyroidism. Growth hormone insufficiency. Syndromes: Turners, Russell-Silver, Noonan's, skeletal dysplasias. Growth impairment e.g. coeliac disease, inflammatory bowel disease or chronic renal failure. Intracranial tumours. Short normal children. Children with short stature. Health promotion: impaired growth may be associated with child abuse or neglect for example. Focus of interest for parents.

Public health aspects: • • •

Secular trend of increasing growth. Linking growth patterns in fetal life and early infancy with adult patterns of disease. Link between height and social circumstances.

37) The following are recognized features of Turner's syndrome: A Low birth weight.

(True)

B Ovarian cysts.

(False)

C Low plasma luteinizing hormone (LH) level.

(False)

D High plasma follicle-stimulating hormone (FSH) level.

(True)

E Lymphoedema.

(True)

Comments: The features of Turner's syndrome are: low birth weight short stature short, webbed neck with low hair line and loose skin folds typical facies (prominent ears, small jaw) high arched palate hypoplastic nails oedema of hands and feet shield-shaped chest cubitus valgus pigmented naevi low IQ hearing impairment coarctation of the aorta renal tract abnormalities streak ovaries high basal LH and FSH.

Pediatrics

16

38) The following conditions present with cyanosis in the newborn: A Tricuspid atresia.

(True)

B Ebstein's anomaly.

(True)

C Atrioventricular septal defect (AVSD).

(False)

D Tetralogy of Fallot.

(True)

E Transient myocardial ischaemia.

(False)

Comments: Cardiac conditions that present with cyanosis in neonates include: 1. Transposition of the great arteries (TGA) 2. Pulmonary atresia 3. Severe pulmonary stenosis 4. Severe tetralogy of Fallot 5. Tricuspid atresia 6. Ebstein's anomaly 7. Total anomalous pulmonary venous drainage (TAPVD) 8. Hypoplastic left heart 9. Truncus arteriosus Babies with AVSD usually become symptomatic after the first 2 weeks of life with symptoms and signs of congestive cardiac failure. Transient myocardial ischaemia is often a feature of perinatal asphyxia. It may cause varying degrees of congestive cardiac failure or may only be evident as temporary ECG changes.

39) Concerning congenital heart disease: A ASD is the commonest malformation at birth

(False)

B Osteogenesis imperfecta is associated with aortic stenosis

(False)

C Ebstein's anomaly is associated with maternal exposure to lithium carbonate

(True)

D congenital complete heart block is usually associated with Anti-Ro antibodies in the mother

(False)

E hypoplastic left heart syndrome is characterised by a large, dilated left ventricle

(False)

Comments: A- VSD is the commonest at 30%, ASD is 10%. B- Aortic regurgitation. C- If exposed in the first trimester. D- In the vast majority of cases the cause is not known but rarely it may be caused by autoimmune disease, particularly Anti-Ro antibodies, in the mother. E- The left sided chambers fail to develop and blood enters the systemic circulation from the right ventricle via the pulmonary artery and a patent ductus arteriosus.

Pediatrics

17

40) Which of the following is true regarding rheumatoid arthritis. A Primarily affects the articular cartilage.

(True)

B Is associated with the HLA antigens DR4 and DW4.

(False)

C Occurs more often in women.

(True)

D The cervical spine is commonly involved.

(False)

E Extra-articular manifestations occur in 20% of patients.

(True)

Comments: Rheumatoid arthritis is a disease of the synovial membrane. It results in joint destruction, pannus formation and periarticular erosion. DR4 and DW4 are associated with diabetes. The female to male ratio is 3:1. The hand, elbows and knees are the commonest joints involved. Extra-articular manifestations occur in 20% and include keratoconjuctivitis sicca, episcleritis, pulmonary nodules, pleural effusions, pericarditis and subcutaneous rheumatoid nodules.

41) Club-foot (talipes equino-varus-tev) deformity in a newborn: A Is most commonly “postural” in origin.

(True)

B Has a higher incidence in babies born by the breech than the vertex.

(True)

C Needs open reduction in most instances.

(False)

D Is more common in Chinese than other races.

(False)

E Occurs in association with spina bifida cystica only if there is accompanying hydrocephalus.

(False)

Comments: Postural talipes is most common and can be passively corrected. Spina bifida cystica refers to meningocele and myelomeningocele; these are associated with other skeletal deformities but not hydrocephalus. It is more common in Polynesians rather than Caucasians and not in Chinese.

42) In examination of the cardiovascular system in childhood: A A venous hum is best heard with the child lying flat

(False)

B Ventricular septal defect may not be clinically detectable in the first few days of life

(True)

C Intercostals recession is useful evidence for a left to right shunt

(True)

D Normal femoral pulses at birth exclude co-arctation of aorta

(False)

E Peripheral oedema is a valuable sign of heart failure in infancy

(False)

Comments: Venous hum is best heard above the Rt clavicle with the child sitting up. VSD not usually detected till after one month of age when there is normal decrease in pulmonary vascular

Pediatrics

18

resistance allowing left to right shunting to take place. Preductal coarctation of the aorta is associated with other cardiac defects and if there is an associated PDA then pulses in the lower extremities will be preserved. Post ductal coarctation is associated with diminished or absent femoral pulses. Right sided venous congestion characteristically causes hepatosplenomegaly in infants, oedema and ascites occur less frequently.

43) A presumed male child of 6 years has apparent hypospadias and no palpable testes. The following would be in favour of the child being female:A buccal mucosa chromatin -ve

(False)

B high urinary 17-ketosteroid excretion

(True)

C pubic hair present

(True)

D abnormally tall stature

(True)

E retarded bone maturation

(False)

Comments: a - females have a barr body b,c,d - suggest CAH and virilization e - not a feature at this age, would expect advanced bone age in a female.

44) Haematological consequences of splenectomy for traumatic rupture of the spleen in a 2 year old include A An increased peripheral white cell count

(True)

B Heinz bodies on the blood film

(True)

C Howell-Jolly bodies on the blood film

(True)

D the development of target cells

(True)

E increased susceptibility to overwhelming septicaemia

(True)

Comments: + siderocytes, inc. plt initially

Pediatrics

19

45) In the management of 2-year child with burns: A Once damp QRS dressings are placed over the burns, they should not be removed till the child reaches the burns unit

(False)

B Lund and Browder chart accurately estimates the percentage of burns

(False)

C A urine output of 0.5 ml/kg/hr is an indicator of adequate hydration

(False)

D Ionotropes may be required in burns > 10% total body surface area

(True)

E Once the burn wounds are dressed, they should be left undisturbed for five days

(False)

Comments: Damp dressings can cause hypothermia in children; therefore, continuous assessment of the child is essential. Lund and Browder chart (rule-of-nines) is useful in adult burns; in children, head makes up 14% of the total body surface area and the legs make up only 14%. Urine output of at least 1ml/kg/hr is required in this age group; 0.5 ml/kg/hr is adequate in adults. Ionotropes such as dopamine may be required if the urine output is not satisfactory. The wound depth should be estimated again after 24-48 hrs, since a partial thickness burn may progress to a full-thickness burn, thus altering the management plan.

46) Which of the following statements is correct regarding osteomalacia?

A Is due to vitamin A deficiency

(False)

B Can cause a distal myopathy

(True)

C May present with pseudo-fractures

(True)

D Serum calcium is increased

(False)

E A bone biopsy would show an increase in mineralized osteoid

(False)

Comments: Osteomalacia is due to vitamin D deficiency T It can result from malabsorption, renal disease (familial hypophosphataemic rickets), chronic renal failure and anticonvulsant therapy (e.g. phenytoin). It often presents with bone pain and a proximal myopathy but a distal myopathy may feature. Serum calcium is low. A bone biopsy would show an increase in the amount of unmineralised osteoid.

47) Which of the following is/are true regarding osteoid osteoma A It is a benign bone tumour

(True)

B The iliac crest is the commonest site involved

(False)

C Presents with severe pain that is typically relieved by aspirin

(True)

D Radiologically appears as an osteosclerotic lesion

(False)

E Local excision or currettage is curative

(True)

Pediatrics

20

Comments: Osteoid osteoma is a benign tumour that mainly affects young adults. The femur and tibia are the commonest bones involved. Pain is often the presenting feature and relief by asprin is almost diagnostic. Local surgery is curative.

48) A complete unilateral facial hemiparesis may be caused by: A

An intracranial tumour

(False)

B Birth injury

(True)

C Phenothiazine toxicity

(False)

D Myasthenia gravis

(False)

E Cerebellar atrophy

(False)

Comments: The facial nerve consists of 2 parts. The larger motor components supplies all the muscles of facial expression, while the smaller part (nervous intermedias) comprises the sensory and parasympathetic branches of taste from the anterior two thirds of the tongue, with efferent fibres to the lacrimal, submaixillary, and sublinguinal salivary glands. Unilateral upper motor neurone lesions (above the level of the ponds) cause weakness more in the lower than in the upper part of the face, since upper facial structures receive bilateral innovation. A unilateral lower motor neurone lesion such as Bell's Palsy, affects the eyes as well. An intracranial tumour can, therefore, cause complete weakness only when both sides are affected. A forceps injury may compress the facial nerve. 49) Which of the following is/are true regarding osteoid osteoma A It is a benign bone tumour

(True)

B The femur and tibia are the commonest bones involved

(True)

C Presents with severe pain that is typically relieved by aspirin

(True)

D Radiologically appears as a radiolucent lesion surrounded by dense bone

(True)

E Local excision or currettage is curative

(True)

Comments: Osteoid osteoma is a benign tumour that mainly affects young adults. The femur and tibia are the commonest bones involved. Pain is often the presenting feature and relief by asprin is almost diagnostic. Xray reveals a radiolucent nidus with a dense rim. Local surgery is curative.

Pediatrics

21

Osteoid osteoma •

Rare, 2:1 M:F

•

Prox phalanx or carpus

•

Pain relieved by aspirin or NSAIDs

•

Xray - eccentric cortical sclerosis & radiolucent nidus

•

Rx curettage using image intensifier

•

13% recurrence

50) Which of the following is true concerning Meningococcal meningitis?

A if it is suspected, benzyl penicillin should be administered prior to hospital admission

(True)

B vaccination is available against all strains of meningococcus

(False)

C sensorineural deafness has been shown to be the most common permanent sequela

(True)

D it has been shown to be more common in those children in contact with cigarette smoke

(True)

E

(True)

Rifampicin has been shown to be the treatment of choice for close contacts

Comments: a - This may be life saving. b - Only against groups A and C. Most patients make a full recovery from meningococcal meningitis but deafness is one of the commoner sequelae. Risk factors include overcrowding, parental smoking. Rifampicin should be administered to close contacts as prophylaxis. (Cornwall Trainers)

60) The following applies to congenital heart defects: A Most deaths occur in the first year of life.

(True)

B There is an increased incidence of heart disease in children who have had rubella.

(False)

C A ventricular septal defect may close spontaneously as a child grows.

(True)

D Down’s syndrome is frequently associated with Fallot’s tetralogy.

(False)

E Tricuspid atresia is the commonest cause of cyanotic heart disease in the first month (False) of life.

Pediatrics

22

Comments: Approximately 51% of deaths occur in infants aged under 1 year and only 7% in age group 1-4. Maternal rubella and not childhood disease is associated with cardiac defects eg pulmonary alular disease, PDA and VSD. Downs syndrome is frequently associated with endocardial cushion defects, which occur in 25% of children and not tetralogy of fallot. In the first month of life transposition of the great arteries is the commonest cause of cyanotic congenital heart disease, but overall tetralogy of fallot is the commonest. 61) The following are common signs of heart failure in infancy: A Breathlessness.

(True)

B Basal crepitations.

(False)

C Tachycardia.

(True)

D Raised JVP.

(False)

E Enlarged liver.

(True)

Comments: Tachypnoea and dyspnoea, tachycardia and hepatomegaly are common clinical signs of heart failure. Cough and wheeze may occur and basal crepitations are uncommon. Raised jugular venous pressure is an unreliable clinical sign in infancy.

62) A chest x-ray showings signs of increased pulmonary blood flow (increased pulmonary plethora) is compatible with a diagnosis of:

A Ventricular septal defect

(True)

B Patent ductus arteriosis

(True)

C Pulmonary tetralogy

(False)

D Fallot’s tetralogy

(False)

E Coarctation of the aorta

(False)

Comments: VSD will cause increased blood flow through the Rt ventricle and pulmonary trunk, as will PDA which connects the left pulmonary artery and descending aorta. In fallots and pulmonary tetralogy there will be decreased flow due to infundibular stenosis. Coarctation, a narrowing of the aorta near the left subclavian vein is associated with normal pulmonary vasculature.

Pediatrics

23

63) Which of the following statements applies to Cleft Palate? A Carries an increased risk of middle ear infections.

(True)

B Has a recognized association with micrognathia.

(True)

C May be associated with maternal anticonvulsant therapy.

(True)

D Is usually repaired before the age of three months.

(False)

E Is associated with subsequent speech disorders.

(True)

Comments: Hearing impairment and repeated ear infections may occur, as well as other craniofacial abnormalities. Anti-convulsants have been associated with cleft lip & palate, congenital heart disease, CNS & skeletal abnormalities. Surgical methods such as bone grafting one are carried out in childhood and are most successful in patients under 10 years of age and as early as 5 to 6 years as the front incisor teeth are erupting. Delayed language and articulation development is common. 64) In children with Down's syndrome: A The infant is usually hypertonic.

(False)

B Asthma is more common.

(False)

C Most mothers are over 35 years of age.

(False)

D Karyotyping is always indicated.

(True)

E Brachycephaly is characteristic.

(True)

Comments: Infants with Down's syndrome (trisomy 21) are commonly hypotonic at birth. Brachycephaly a consistent clinical feature. Owing to cases of Down's syndrome where a translocation has occurred, karyotyping should always be carried out in order to assess the recurrence risk in parents and other family members.

65) A 3-year-old child presents to hospital with a 10-day history of abdominal pain and bloody diarrhoea. On examination the child appears anaemic and jaundiced. The following laboratory findings are consistent with a diagnosis of haemolytic uraemic syndrome (HUS):

A Positive Coombs' test.

(False)

B Normal osmotic fragility test.

(True)

C Low reticulocyte count and anaemia.

(False)

D Increased fibrin degradation products.

(True)

E Raised lactate dehydrogenase levels in the serum.

(True)

Pediatrics

24

Comments: HUS is the most common cause of acute renal failure in an otherwise healthy child. It presents with bloody diarrhoea that may have been present for 1-3 weeks. E. coli 0157:H7 is the most commonly implicated organism. Anaemia results from haemolysis of red blood cells (RBC) in the microvasculature. Laboratory findings include: reticulocytosis, unconjugated hyperbilirubinaemia, increased lactate dehydrogenase (released from RBC), decreased haptoglobulins, a negative Coombs' test and normal red cell enzymes and osmotic fragility. A neutrophil leucocytosis usually occurs. If the leucocytosis is > 20 x 10E9/l, there is a poorer prognosis.

66) In bulimia nervosa: A Male cases do not occur.

(False)

B Weight is usually normal.

(True)

C Dental problems are a recognized association.

(True)

D Diabetes mellitus is a recognized association.

(True)

E The presence of amenorrhoea indicates a worse prognosis.

(True)

Comments: Bulimia nervosa is an eating disorder (more common than anorexia nervosa) in which an intense preoccupation with food and weight is accompanied by episodes of binge eating and self induced vomiting. Over 90% of cases are female. Recurrent vomiting may cause erosion of dental enamel and a worse prognosis occurs in patients with menstrual irregularities, biochemical disturbances and concurrent depressive.

67) Oral rehydration therapy: A works by passive absorption of glucose;

(False)

B Is effective in 50% of moderately dehydrated infants.

(False)

C Should not be used in hypernatraemic dehydration.

(False)

D Contains 5 mmol/l of potassium.

(False)

E Can be made up safely at home using salt and sugar.

(False)

Comments: Oral rehydration therapy works as glucose is actively absorbed across the lumen of the intestine in a cotransport system with sodium. It is effective in up to 90% of infants in the UK with moderate dehydration. Oral rehydration therapy is the treatment of choice for hypernatraemic dehydration as there is a more gradual correction of electrolyte imbalances than using intravenous therapy. All oral rehydration solutions contain potassium concentrations of 20 mmol/l. Solutions made up using home ingredients should be discouraged because of the possibilities of making hypertonic solutions.

Pediatrics

25

68) A five year-old child presents with a high swinging fever. A diagnosis of systemic onset juvenile chronic arthritis (JCA) is more likely if the following features are present: A The child is female.

(False)

B HLA studies show that the child is HLA-B27.

(False)

C The child is rheumatoid factor positive.

(False)

D Iridocyclitis is present.

(False)

E Hepatospenomegaly is present.

(True)

Comments: Systemic onset JCA is typified by a persistent, high swinging fever, a 'rheumatoid' rash, hepatosplenomegaly, lymphadenopathy and pleuritis. It has an equal sex distribution. HLA associations with the condition are not yet know. HLA-B27 is seen in about 90% of children seen with juvenile ankylosing spondylitis. Children with systemic onset JCA are rheumatoid factor negative and do not develop iridocyclitis. In pauciarticular JCA up to 50% of those who are antinuclear factor positive will develop chronic iridocyclitis.

69) The following features make a diagnosis of ulcerative colitis more likely than one of Chrohn's disease:

A Perianal lesions.

(False)

B Crypt abscesses on biopsy.

(True)

C Granulomata.

(False)

D Pyoderma gangrenosum.

(True)

E Presence of a fistula.

(False)

Comments: Perianal lesions T (tags, abscess, and fistulae) and the development of a fistula are highly suggestive of Crohn's disease (terminal ileum). Histological features of ulcerative colitis (colon) include mucosal disease with crypt abscesses, decreased goblet cells and an infiltration of polymorphs. Granulomata are not a feature of ulcerative colitis but pyoderma gangrenosum does occasionally develop. (non-caseating granulomas) Tin the biopsy of chrohns disease. 70) Intra-uterine infection may result in: A Short stature.

(True)

B Intraventricular haemorrhage.

(False)

C Retinal vein thrombosis.

(False)

D Patent ductus arteriosus.

(True)

E Mental handicap.

(True)

Pediatrics

26

Comments: Intrauterine infection results in prematurity, growth failure and cerebral palsy. Intraventricular haemorrhage is inversely proportional to birth weight and gestational age and related to neonatal sepsis. Retinal vein thrombosis is not associated. Patent ductus arteriosus can follow prematurity or rubella infection.

71) Acute osteomyelitis in children: A Is commonly caused by Streptococcus pyogenes infection

(False)

B Usually commences in the epiphysis

(False)

C Blood cultures are always positive

(False)

D A sequestrum and involucrum are classical radiological features

(False)

E Joint fluid aspiration may be necessary to make a diagnosis

(True)

Comments: Acute osteomyelitis is commonly caused by Staphyloccus aureus infection. It may be acquired by haematogenous route, direct skin puncture following injury or from infection spreading from adjacent soft tissues. It usually commences in the metaphyseal region of long bones, but at later stages could affect the growth plate and epiphyseal cartilage. The child with acute osteomyelitis usually presents after several hours of pain, malaise and fever. The child refuses to walk or to move an affected limb. There is invariably local tenderness over the inflammation; local redness, swelling and oedema are late signs, but joint movement is often painful. Blood cultures may be positive in about 60% of cases. A sequestrum and involucrum are features of chronic osteomyelitis; a sequestrum is a necrotic nidus of bone within a focus of chronic osteomyelitis while an involucrum is a cloak of new bone produced by the periosteum around the infection. If the child is refusing to move the limb/joint, then a joint fluid aspiration may be necessary to distinguish acute osteomyelitis from septic arthritis. 72) The following statements regarding sudden infant death syndrome (SIDS) are correct: A The incidence is equal for infants born permaturely and at term.

(False)

B It occurs more commonly in lower socio-economic groups.

(True)

C If parents smoke, there is a two-fold increased risk of a baby dying as a result of SIDS.

(True)

D SIDS does not occur after 6 months of age.

(False)

E Siblings of children who suffered SIDS have a five-fold increased chance of dying from SIDS.

(True)

Comments: If babies are left to sleep in the prone position there is an increased risk of SIDS. There is an increased incidence of SIDS in premature infants, low-birth-weight infants and siblings of SIDS victims. It is more common in lower socio-economic groups. Parental smoking (maternal>paternal) is also a risk factor. Peak incidence is around 3-4 months and most cases occur before 6 months of age although it has been reported up to 1 year.

Pediatrics

27

73) Small-for-gestational-age infants are more likely than appropriate-for-gestationalage infants to have: A Hypoglycaemia.

(True)

B Hypocalcaemia.

(True)

C Respiratory distress syndrome.

(False)

D Congenital malformations.

(True)

E Persistent fetal circulation.

(True)

Comments: Small for gestational age fetuses are more likely than normal newborn infants to encounter several problems in the neonatal period. These include: hypothermia, hypoglycaemia due to reduced glycogen stores, impaired gluconeogenesis and relative hyperinsulinism, hyperglycaemia, respiratory difficulties (meconium aspiration and chest infections), polycythaemia and persistent fetal circulation.

74) The following are features of premature thelarche: A Peak onset between 6 months and 2 years.

(True)

B Association with Turner Syndrome

(False)

C Asymmetrical breast enlargement.

(True)

D Associated growth spurt.

(False)

E Areola pigmentation.

(False)

Comments: Premature thelarche is common, with a peak incidence between 6 months and 2 years. It may be asymmetrical, and is differentiated from true precocious puberty by the absence of pubic hair development and a growth spurt. It is usually self-limiting. 75) Left axis deviation is seen on the ECG in the following conditions: A Patent ductus arteriosus.

(False)

B Ebstein's anomaly.

(False)

C Atrioventricular canal defects.

(True)

D Large ventricular septal defect.

(False)

E Tetralogy of Fallot.

(False)

Comments: Left axis deviation J is also seen in tricuspid atresia, Atrioventricular canal defects.

Pediatrics

28

76) Hypersecretion of gastric acid is associated with: A Duodenal ulcer.

(True)

B Vagotomy.

(False)

C Short bowel syndrome.

(True)

D Zollinger-Ellison syndrome.

(True)

E Ranitidine.

(False)

Comments: Gastric acid secretion is facilitated by parasympathetic stimulation via the vagus nerve in addition to local hormonal mechanisms mediated by gastrin. Duodenal ulceration is usually associated with hypersecretion of gastric acid whereas gastric ulceration often occurs with subnormal levels. Ranitidine inhibits gastric acid secretion by H2 receptor antagonism. It is often useful in short bowel syndrome as hypergastrinaemia occurs in many cases leading to excessive gastric acid secretion. In Zollinger-Ellison syndrome there is hypersecretion of the gastrin-secreting cells of the antrum of the stomach. This leads to increased gastric acid secretion causing multiple peptic ulcers. 77) A six month old baby presents with a generalized rash, affecting her trunk and extremities virtually equally. The diagnosis includes: A Measles in mild form.

(False)

B Meningococcal septicaemia.

(True)

C Pseudomonas septicaemia.

(True)

D Haemophilia.

(False)

E Giant urticaria.

(False)

Comments: The measles rash starts about 14 days after exposure and appears as a generalized macular eruption on face, neck and spreads over 3 days. Meningococcal septicaemia causes a generalized non-blanching purpuric eruption. Pseudomonas and other gram-negative septicaemia can cause a rash, eccymoses and petechiae. Haemophilia is not associated with generalized rash. Giant urticaria.is characterized by recurring attacks of transient edema suddenly appearing in areas of the skin or mucous membranes and occasionally of the viscera, often associated with dermatographism, erythema, and purpura.

Pediatrics

29

78) Stiff neck in a young child occurs in: A acute poliomyelitis

(True)

B retropharyngeal abscess

(True)

C rickets

(False)

D Still’s disease

(True)

E Vitamin A deficiency

(False)

Comments: 1.due to the meningitic component in acute poliomyelitis 2.a well recognized cause. The neck is hyperextended. 4.due to the involvement of the cervical spine in the arthritis of Still’s disease

79) The following conditions are associated with bilateral pulmonary hypoplasia: A Preterm prelabour rupture of membranes.

(True)

B Diaphragmatic hernia.

(True)

C Maternal smoking.

(False)

D Myotonic dystrophy.

(True)

E Renal agenesis.

(True)

Comments: The following are associated with pulmonary hypoplasia: Oligohydramnios - renal aplasia/severe dysplasia (Potter's syndrome), - premature rupture of membranes, - postamniocentesis. Compression of lung - congenital diaphragmatic hernia, - cystic adenomatoid malformation, - pleural effusion, - small chest syndromes. Neuromuscular disease - spinal muscular atrophy, - myotonic dystrophy. 80) Regarding osteosarcomas A Affects the epiphyses of long bones

(False)

B Are most commonly seen around the knee and in the proximal humerus

(True)

C Haematogenous spread can result in pulmonary metastases

(True)

D Is exclusively a disease of adolescence and early adult life

(False)

E X-ray shows a 'sunburst' appearance due to soft tissue involvement

(True)

Comments: Osteosarcomas affect the metaphyses of long bones. They are most commonly seen around the knee and in the proximal humerus. They often occur in young adults but are also seen in the elderly in association with Paget's disease. They usually present as bone pain and a palpable lump. X-ray shows periosteal elevation (Codman's triangle) and a 'sunburst' appearance due to soft tissue involvement. Early haematogenous spread occurs and the 5year survival rate is approximately 50%.

Pediatrics

30

81) Koplik's spots appear: A two days before the rash of measles

(True)

B opposite incisor teeth

(False)

C only when fever is over 39°C

(False)

D as red papules on the dorsum of the hands

(False)

E fluorescent under Wood's light

(False)

Comments: Classically Koplik's spots are pathognomonic of measles being found opposite the premolars two days prior to the development of the rash. 82) Causes of chronic rhinitis include: A Tetanus

(False)

B Dysmotile cilia syndrome

(True)

C Foreign body

(True)

D Henoch-SchXnlein purpura

(False)

E Wegener granulomatosis

(True)

Comments: Chronic nasal discharge with or without acute exacerbations may reflect an underlying disturbance such as: • •

Local: Nasal polyps, chronic sinusitis, chronic infected adenoids, deviated septum, foreign bodies, nasal diphtheria. Generalised: Cystic fibrosis, dismotile cilia syndrome, allergy, syphilis, Wegener granulomatosis, immune deficiency, hypothyroidism.

The commonest symptom is chronic nasal discharge with foul odour. A bloody discharge suggests a foreign body, nose picking, syphilis, or diphtheria.

83) Congenital hypothyroidism: A Has an incidence of some 1:1000 births

(False)

B Is screened for at birth by testing Thyroxine levels in the blood

(False)

C If untreated may be a cause of precocious puberty

(True)

D May present as “short stature”

(True)

E Is associated with prolonged jaundice in the neonatal period

(True)

Pediatrics

31

Comments: Congenital hypothyroidism has an incidence of 1 in 4000 births. It is screened for by measuring TSH levels. If untreated precocious puberty may occur, however growth and skeletal maturation is impaired. It may present as growth failure and short stature. It is rarely associated with prolonged conjugated hyperbilirubinaemia in neonates.

84) Truncal (sensory) ataxia is recognized in: A Extrapyramidial diseases

(False)

B Lesions of the cerebellar vermis

(True)

C Benzodiazepine overdose

(True)

D Middle cerebral artery occlusion

(False)

E Phenytoin

(True)

Comments: The smooth accurate performance of purposeful movements require intact sensory and motor functions as well as efficient control by higher centres. Any lesion causing weakness can be accompanied by clumsiness, but incoordination is particularly prominent in sensory and cerebellar ataxia. Sensory ataxia results from defective propreception, and can be mitigated by visual control. It is, therefore, exacerbated when the eyes are closed. Cerebellar ataxia - peripheral propreceptive information is fed to the posterior lobe of the cerebellum, which is also connected to the motor cortex. This incoordination is, therefore, not susceptible to visual compensation. Tests of coordination include: 1. 2. 3. 4. 5.

Finger/nose test. Finger to finger test. Intention tremor. Heel to knee test. Dysdiadochokinesis.

Phenytoin gives rise to nystagmus as well as ataxia, and this is, therefore, central in origin. The effects of Diazepam are also centrally mediated with associated drowsiness.

85) Regarding osteosarcomas A Affects the epiphyses of long bones

(False)

B Are most commonly seen around the knee and in the proximal humerus

(True)

C Haematogenous spread can result in pulmonary metastases

(True)

D Is exclusively a disease of adolescence and early adult life

(False)

E X-ray shows a 'sunburst' appearance due to soft tissue involvement

(True)

Pediatrics

32

Comments: Osteosarcomas affect the metaphyses of long bones. They are most commonly seen around the knee and in the proximal humerus. They often occur in young adults but are also seen in the elderly in association with Paget's disease. They usually present as bone pain and a palpable lump. X-ray shows periosteal elevation (Codman's triangle) and a 'sunburst' appearance due to soft tissue involvement. Early haematogenous spread occurs and the 5year survival rate is approximately 50%.

86) Hypertension in childhood typically complicates A Post-streptococcal glomerulonephritis

(True)

B Coarctation of the aorta

(True)

C Minimal change nephrotic syndrome

(False)

D Appendicitis

(False)

E Peptic ulceration

(False)

Comments: Hypertension in childhood is commonly secondary due to renal disease (Glomerulonephritis), heart disease (coarctation) or endocrine disease (Phaeochromocytoma). Minimal change disease is not complicated by hypertension and typically resolves with no sequelae.

87) Regarding Salter-Harris classification for bone injuries in children: A This classification is for fractures through the metaphysis and the diaphysis

(False)

B Growth arrest is common in Salter-Harris type I injury

(False)

C Salter-Harris type IV is the commonest form of growth plate injury

(False)

D In Salter-Harris type V, there is compression of the epiphysis leading to growth arrest

(False)

E Accounts for 120-140 bpm, are common. Hepatomegaly is a reliable indicator of effectiveness of therapy and is common. Frank pulmonary oedema is uncommon and raised JVP, though useful in older children is not a reliable clinical sign in infants.

173) Features of Bartter's syndrome are: A Hypertension.

(False)

B Low urinary chloride.

(False)

C Hyperreninaemia.

(True)

D Hyperaldosteronism.

(True)

E Juxtaglomerular hypoplasia.

(False)

Comments: Bartter's syndrome presents with failure to thrive, muscle weakness, polyuria and normal blood pressure. Biochemical features include hypokalaemia (usually 40 degrees, diffuse macular rash which later desquamates, tachynoea and oliguria. These signs usually develop on the third or fourth day

Pediatrics

98

after sustaining the scald/burn and are associated with a drop in haemoglobin and white cell count. It is caused due to a toxin produced by Staphylococcus aureus phage type 29/52. Late complications include irritability, cerebral oedema, convulsions and coma. Treatment should be prompt with control of temperature by vasodilatation, reduction of cerebral oedema, if necessary by hyperventilation, and the administration of whole blood, immunoglobulins and antibiotics.

Associations have been established between: A Adrenal hyperactivity and excess skin melanin

(False)

B Black eye and haemosiderin

(True)

C Calcification around renal tubules and excess of vitamin B

(False)

D Fibrocystic disease of the pancreas and mucous secretion defects

(True)

E Renal stones and cystinuria

(True)

Comments: Excess skin melanin can be the result of hypoadrenalism and high circulating ACTH and POMC. Haemosiderin deposition occurs causing pigmentation. Excessive B6 may cause symptoms of a peripheral neuropathy, but calcification is not associated. Cystic fibrosis is associated with defective mucous secretion hence complications such as bronchiectasis and meconium ileus. Cystinuria is associated with nephrolithiasis.

Mastocytosis: A is characterized by aggregates of tissue mast cells in the epidermis.

(False)

B commonly presents with pruritis.

(True)

C may be exacerbated by aspirin.

(True)

D takes the form of a solitary mastocytoma in about 50% of cases.

(False)

E most commonly takes the form of urticaria pigmentosa.

(True)

Comments: Mastocytosis encompasses a spectrum of disorders (mastocytoma, urticaria pigmentosa, diffuse mastocytosis and systemic mastocytosis) with aggregates of mast cells in the dermis. Symptoms result from the release of histamine, which produces a flush, tachycardia, headache, diarrhoea, hypotension, syncope and respiratory distress. It is exacerbated by hot baths, vigorous rubbing, aspirin, codeine, morphine, atropine and polymyxin B. About 10% have a solitary lesion, i.e. mastocytoma. From Hannam et al. MRCP (Paediatrics) Part 1 MCQs. page 61 © WB Saunders. Reproduced with permission.

The following statements regarding dental development are correct: A calcification of the teeth starts in the seventh month of fetal life.

(True)

Pediatrics

99

B in most children, deciduous teeth have started to erupt by 12 months.

(True)

C the first permanent teeth to erupt are molars.

(False)

D eruption of deciduous teeth can be delayed in hypothyroidism.

(True)

E calcification of permanent teeth starts at 2 years.

(False)

Comments: Calcification of teeth begins in the 7th month of fetal life. Initially this is seen in the deciduous teeth until shortly before term when calcification of permanent teeth begins. In most infants eruption of deciduous teeth has started by 6 months although this can be delayed by factors such as malnutrition and hypothyroidism. From Hannam et al. MRCP (Paediatrics) Part 1 MCQs. page 29 © WB Saunders. Reproduced with permission.

In a patient with a head injury leading to a six hour coma, the following factors will affect CNS outcome: A Right parietal injury compared to left parietal injury

(True)

B Age

(True)

C Presence of chest injury

(True)

D Hemiplegia

(True)

E Glasgow coma scale at 24 hours

(True)

Comments: Major insults to the brain may result from contusion (particularly to the frontal lobes or inferolateral portion of the temporal lobes) a penetrating injury, the presence of an intracerebral haemorrhage, and diffuse axonal injury due to shear forces, all of which may produce cerebral oedema. The incidence of space-occupying intracranial haematoma is less frequent in children than in adults, but the presence of diffuse cerebral swelling due to diffuse axonal injury is more frequent, particularly in a severe paediatric head injury. The clinical findings depend on the site and nature of the injury and on the degree of cerebral oedema. The development of additional neurologic signs following admission to the hospital implies increased cerebral oedema; an expanding intracerebral, subdural, or epidural haematoma; or compromised cerebral blood flow secondary to vasospasm. Initial signs associated with a poor prognosis include fixed and dilated pupils, apnoeic breathing, decorticate posturing, and a Glasgow Coma Scale score revised for children of less than 5. Some children are found to have cardiorespiratory arrest and hypotension at the site of an accident following a head injury, which is unrelated to acute blood loss. Cervical spine radiographs often show an injury to the high cervical spine, and postmortem examination shows intraparenchymal haemorrhage or laceration. Although this type of injury is uniformly fatal, it serves as a reminder to consider a spinal cord injury in every child with a severe head injury until appropriate radiographic studies are completed. The most important determinant of neurologic and intellectual recovery in the head-injured child is the duration of coma. If the child survives the immediate consequences of the head injury and recovers from coma within 14 days, the likelihood of normal or near-normal cognitive and neuromotor function is extremely favourable. The reasons for optimism in the child compared with the adult is the contention that the former's brain is more "plastic" than the

Pediatrics100

adult's and generally recovers more completely in a shorter time. However, infants less than 2 year of age with major brain trauma have a uniformly poor prognosis compared with older children, perhaps as the result of immature autoregulation of the cerebral blood vessels, the greater susceptibility of the incompletely myelinated brain to irreversible injury, and the fact that open cranial sutures permit greater distortion among the meninges, cerebral vessels, and the underlying brain. The most sensitive and specific indicator of neurologic recovery during the acute and early stages of a head injury is an evaluation with somatosensory evoked potentials. Late post-traumatic seizures tend to develop within 2 year of the initial insult. Posttraumatic epilepsy is more likely to occur if the original trauma to the brain was severe and the dura disrupted. Copyright © 2002 Dr Colin Melville

Which of the following is true of patent ductus arteriosus: A Functional closure in the normal infant term occurs within the first 24 hours.

(True)

B Pulmonary atresia is a duct-dependent malformation.

(True)

C It is possible to keep the ductus open by indomethacin.

(False)

D Its incidence is lower in preterm babies.

(False)

E Its incidence is lower in children in high altitude communities.

(False)

Comments: The physiological function of the ductus arteriosus in the fetus is to divert blood away from lungs to the placenta, constriction occurs in the first 24 hours of life in response to increased environmental oxygen. In order to maintain life some cardiac malformations such as pulmonary atresia, hypoplastic left ventricle and interrupted aortic arch require the patency of a wide PDA after birth. Indomethacin causes duct closure through an anti- prostaglandin effect. Incidence of PDA is greater in prefer infants and birth in a high altitude environment is associated with greater risk.

At birth a baby has A stepping reflex

(True)

B its own IgG

(False)

C its own IgM

(False)

D positive Babinski response

(True)

E nasal respiration

(True)

Comments: 2. has maternal IgG which can cross the placenta 3. IgM does not cross the placenta, and does not reach adult levels until the infant is 2-5 months old. 4. upgoing plantars can be normal up to the age of one year

Pediatrics101

A newborn infant has ambiguous genitalia. The following statements are true: A assignment of gender is primarily dependent on karyotype.

(False)

B if gonads are palpable they are likely to be testes.

(True)

C the infant should be raised as a boy in all cases of male pseudohermaphroditism.

(False)

D if the karyotype is 46XX, 21-hydroxylase deficiency is the most likely diagnosis.

(True)

E gonads that are discordant for the assigned gender should be removed.

(True)

Comments: In cases of ambiguous genitalia the determination of sex for rearing should be primarily determined by the feasibility of anatomic reconstruction and appropriate hormonal treatment rather than karyotype. The broad categories of diagnosis are female pseudohermaphroditism (most commonly secondary to 21-hydroxylase deficiency), male pseudohermaphroditism or true hermaphroditism (rare). Male pseudohermaphrodites may have disorders of testosterone synthesis or partial end organ resistance to testosterone (incomplete testicular feminization). The latter should never be raised as males as they never virilize. Gonads that are either discordant for the assigned gender or dysgenetic are surgically removed to reduce the risk of later malignancy. From Hannam et al. MRCP (Paediatrics) Part 1 MCQs. page 55 © WB Saunders. Reproduced with permission

Papilloedema in pre-school children can be caused by: A Hyperthyroidism

(False)

B Pseudohypothyroidism

(False)

C Nephrotic syndrome

(False)

D TB meningitis

(True)

E Posterior fossa tumour

(True)

Comments: Papilloedema is swelling of the optic nerve head due to raised intracranial pressure. If the same appearance arises from an intrinsic lesion of the optic nerve, it is known as papillitis. The ophthalmoscopic appearances are identical of these 2 conditions. Papilloedema of recent onset usually produces little or no change in visual acuity, and papillitis is associated with a severe decrease in vision (central scitoma). Causes in childhood include: hydrocephalus, brain tumours, cerebral abscess, intracranial bleed. Copyright © 2002 Dr Colin Melville

A 5-year-old boy refuses to go to school. The following statements regarding school phobia are correct:

Pediatrics102

A it is more common in boys than girls.

(False)

B it is associated with a low IQ.

(False)

C somatic complaints often accompany the phobia.

(True)

D truancy is common in children who have school refusal.

(False)

E school phobia has a better prognosis in early childhood than in adolescence.

(True)

Comments: In truancy, children leave home but do not arrive at school or leave early. It is commonly associated with a low IQ and is more common in boys than girls, unlike school phobia which has an equal sex ratio. Often in children suffering from school phobia there are functional symptoms present such as abdominal pain or headaches. The condition carries a better prognosis if treated and recognized at an earlier age. From Hannam et al. MRCP (Paediatrics) Part 1 MCQs. page 175 © WB Saunders. Reproduced with permission.

Recognised causes of hypertension in childhood include A chronic glomerulonephritis

(True)

B renal vein thrombosis

(True)

C neuroblastoma

(True)

D coarctation of the aorta

(True)

E congenital adrenal hyperplasia

(True)

Comments: Investigation of hypertension in childhood must always include examination of the urine for albumin, red cells and casts. This will exclude chronic glomerulonephritis. In addition urine should be collected to detect the presence (or absence) of catecholamines. Hypertension can be seen in congenital adrenal hyperplasia in both 11-beta-hydroxylase and 17 hydroxylase deficiencies. From Hannam et al. MRCP (Paediatrics) Part 1 MCQs. page 3 © WB Saunders. Reproduced with permission. Which of the following is true of isolated ventricular septal defects in childhood A A loud systolic murmur on Day 10 indicates a large defect.

(False)

B A loud first heart sound indicates pulmonary hypertension.

(False)

C Heart failure uncommonly presents in the first month of life.

(True)

D There is no need to give ampicillin before dental extraction.

(False)

E Surgical closure is needed if the defect does not close by the age of five.

(False)

Comments:

Pediatrics103

Small defects may present with loud murmurs. A loud pulmonary component of the second heart sound indicates pulmonary hypertension. The first clinical signs of a VSD are not apparent usually till after 1 month. Prophylactic antibiotics are mandatory before dental extraction. Over 50% of the defects close spontaneously.

Breath holding attacks: A Are commoner over the age of 3 ½ years.

(False)

B Can be confused with a generalised convulsion.

(True)

C May be precipitated by a minor injury.

(True)

D Should be treated with sedatives.

(False)

E Are never fatal.

(True)

Comments: The attacks are commonest in infants and younger children, typically occur during crying and emotional outbursts and there is voluntary holding of breath followed by cyanosis and loss of consciousness. The can be precipitated by crying, minor injuries and convulsive movements develop following cyanosis. The cyanosis always precedes the convulsions. They are self resolving and not fatal.

A baby with left congenital diaphragmatic hernia presented with dyspnoea and cyanosis one hour after birth. Which of the following features would be expected? A The abdomen would also be distended.

(False)

B Both apex beat and trachea are likely to be deviated to the right.

(True)

C The first resuscitative measure should be to supply oxygen and assist ventilation with a face mask.

(False)

D The most common site of the diaphragmatic defect is posterolaterally.

(True)

E The prognosis would be better if the baby had presented later at 48 hours after birth.

(True)

Comments: The displacement of abdominal contents into the thoracic cavity occurs.The stomach and abdominal contents cause displacement of thoracic structures to the right, and lung collapse ensues. A diaphragmatic hernia is a surgical emergency. The abdominal organs must be replaced into the abdominal cavity, and the opening in the diaphragm repaired. Invasive ventillation is necessary immediately after birth until the infant recovers from surgery. Some infants are placed on ECMO (extracorporeal membrane oxygenation) which is a heart/lung bypass machine which enables the lungs to expand after surgery.

Left ventricular hypertrophy can occur in:

Pediatrics104

A Tetralogy of Fallot.

(False)

B Ventricular septal defect.

(True)

C Atrial septal defect.

(False)

D Patent ductus arteriosus.

(True)

E Coarctation of aorta.

(True)

Comments: Right ventricular hypertrophy occurs with tetralogy of Fallot and left ventricular hypertrophy occurs with a moderate sized VSD, PDA and coarctation. Right atrial enlargement occurs with ASD.

A right sided lesion of the 6th cranial nerve: A Causes diplopia when looking to the right.

(True)

B Causes a convergent strabismus.

(True)

C The false image is to the left of the true image..

(False)

D The false image is parallel to the true image.

(False)

E The images separate wider when looking to the left.

(False)

Comments: Abnormalities of III, IV and VI (RL6SO4)result in defects with: Eyelids, ptosis, palpebral fissures. Pupils, size, shape, symmetry and reflexes. Eye movements, diplopia nystagmus. The abducent (VI) arises from the lower pons anterior to the 4th ventricle. It runs a long course intracranially, and is liable to stretching. It supplies the lateral rectus. It contains both parasympathetic and sympathetic fibres. The median longitudinal bundle connects III, IV and VI with vestibular inputs, and co-ordinates lateral gaze. Copyright © 2002 Dr Colin Melville

A neonate is born with a supraventricular tachycardia which had been diagnosed antenatally. It has a blood pressure of 30/10, an enlarged liver and a pulse rate of 220/min. The following statements regarding management of the baby are correct: A vagatonic manoeuvres are not appropriate in this situation.

(False)

B adenosine is the intravenous treatment of choice.

(True)

C DC cardioversion at 10 J/kg may be used.

(False)

D intravenous verapamil and propranolol may be of use in this situation.

(False)

E in one-third of cases of SVT there is an underlying congenital cardiac lesion.

(True)

Pediatrics105

Comments: Although in this situation it is unlikely that vagal stimulation would stop the arrhythmia, it is worth attempting unilateral carotid massage even when there is evidence that the infant is in a haemodynamically compromised state. Adenosine at a starting dose of 50 mcg/kg can be very effective at rapidly converting an SVT thythm to a normal sinus rhythm. It works by slowing conduction through the A-V node which interrupts re-entry circuits. DC cardioversion at 0.5-1 J/kg can be extremely effective in returning the heart to a sinus rhythm, especially when the infant is in cardiac failure. Verapamil and propranolol should never be used together as hypotension and asystole can be precipitated. Verapamil is not recommended in children. From Hannam et al. MRCP (Paediatrics) Part 1 MCQs. page 7 © WB Saunders. Reproduced with permission.

Infants of diabetic mothers are at increased risk from: A Hypocalcaemia

(True)

B Anaemia

(False)

C Jaundice

(True)

D Shoulder dystocia

(True)

E Congenital abnormalities

(True)

Comments: Hypoglycaemia, hypocalcaemia and hyperbilirubinaemia are common metabolic derangements. Polycythaemia rather than anaemia is associated. Shoulder dystocia results from the increased birthweight and length of each infant. Malformations of organ systems are increased 2-3 fold. Caudal regression syndrome i.e. hypoplasia of lower segment of the body and congenital heart disease are examples.

The following statements regarding Wilson's disease are correct: A low caeruloplasmin levels are diagnostic of Wilson's disease.

(False)

B Wilson's disease can present as a deterioration in school performance.

(True)

C colchicine challenge is a useful diagnostic test.

(False)

D total serum copper levels are always high.

(False)

E siblings of a child with this condition have a 1 in 4 chance of having Wilson's disease.

(True)

Comments: Wilson's disease is inherited as an autosomal recessive condition. It is essential that asymptomatic siblings and cousins of children with the condition are fully investigated. Although most children with Wilson's disease have a low caeruloplasmin level, levels can be normal in between 4 and 20% of patients. Low values can also be found in heterozygotes for the condition as well as in nephrotic syndrome, severe malabsorption, fulminant hepatitis, chronic active hepatitis and tyrosinaemia. The pathognomonic sign of Wilson's disease is the presence of a Kayser-Fleischer ring which can be seen on slit lamp examination. As well as

Pediatrics106

hepatic manifestations of the disease, Wilson's disease can present with neurological or psychological symptoms. Urinary copper excretion, pre-and post-penicillamine challenge, should be measured in any patient with Kayser-Fleischer rings. Total serum copper can be normal, high or low. From Hannam et al. MRCP (Paediatrics) Part 1 MCQs. page 159 © WB Saunders. Reproduced with permission. Surfactant production: A takes place in type 1 pneumocytes.

(False)

B can be detected at 20 weeks gestation.

(True)

C is decreased by maternal corticosteroid administration.

(False)

D is increased by intrauterine growth retardation (IUGR).

(True)

E is decreased by maternal opiate abuse.

(False)

Comments: Surfactant is produced by type 2 pneumocytes. Although small amounts may be detected as early as 20 weeks gestation, physiologically significant quantities may not be produced until 30-32 weeks. Surfactant production may be accelerated by several maternally ingested substances including glucocorticoids and opiates. IUGR and other causes of intrauterine 'stress' may also enhance production of surfactant. From Hannam et al. MRCP (Paediatrics) Part 1 MCQs. page 169 © WB Saunders. Reproduced with permission.

The following suggest a diagnosis of epiglottitis rather than croup: A Onset over days

(False)

B Severe barking cough

(False)

C Temperature of 38°C

(False)

D A toxic, ill-looking child

(True)

E Drooling of saliva

(True)

Comments: Croup tends to have an onset over days, with preceding coryza. The child remains able to drink and is not drooling. The child does not look particularly unwell and the fever is low grade. The symptoms of cough and stridor are relatively marked and the voice is hoarse. With epiglottitis, the onset is rapid with no history of preceding coryza. There may be an absence of Hib immunisation. The child is toxic and ill, is drooling saliva, and is unable to drink, often with a high fever of above 38.5°C. The child is reluctant to speak with a soft whispering stridor and absent or slight cough. Copyright © 2002 Dr Colin Melville

Most children with Down’s syndrome:

Pediatrics107

A Require institutional care

(False)

B Require special schooling

(True)

C Have associated epilepsy

(False)

D Cannot be trained to live independently

(True)

E Develop autistic symptoms

(False)

Comments: Most children with Down’s syndrome can be brought up at home, however due to the high frequency of mental retardation they cannot attend normal classes or be trained to live independently in adulthood. Epilepsy risk is not greater in these individuals. Autism occurs in about 10% of cases.

The following statements regarding the diagnosis and management of iritis are correct: A the pupil of the affected eye will be dilated and irregular.

(False)

B if the eye is painful a diagnosis of iritis is unlikely.

(False)

C the most common systemic association with iritis in children is with juvenile rheumatoid arthritis.

(True)

D atropine can be used in therapy.

(True)

E retinal detachment is a recognized complication.

(True)

Comments: Inflammation of the anterior part of the uveal tract can present either as iritis or cyclitis (ciliary body inflammation). Iritis presents with photophobia, pain, blurred vision and a small and irregular pupil. Iritis is most commonly associated with juvenile rheumatoid arthritis although it is also seen in toxoplasmosis, histoplasmosis, sarcoidosis, tuberculosis, mumps, measles and herpes simplex infection. A cycloplegic such as atropine is given to dilate the pupil and relieve iris spasm. Topical corticosteroids are also indicated as long as a viral infection is not thought to be present. Complications such as retinal detachment and glaucoma are sometimes seen. From Hannam et al. MRCP (Paediatrics) Part 1 MCQs. page 97 © WB Saunders. Reproduced with permission.

In the management of burns in a specialised burns unit: A In a fit 34-year old male with full-thickness burns involving 55% total body surface area, late debridement and grafting is recommended

(False)

B Skin grafting is absolutely contraindicated in the presence of Enterococcus faecalis in the wound

(False)

C Meshed skin grafts achieve superior cosmetic results compared to unmeshed skin grafts

(False)

D Autografting (during the first surgery) is always possible in burns involving up to 60% total body surface area

(False)

Pediatrics108

E Scalds always cause more skin damage (in terms of burn depth) than chemical burns

(False)

Comments: In a patient with full-thickness burns, early excision of all burned skin is recommended. Grafting, if not possible, could be done at a later time. Enterococcus faecalis is a common skin contaminant in burns and its colonisation does not preclude to successful skin grafting. Unmeshed skin grafts achieve superior cosmetic results compared to meshed skin grafts. Meshed grafts are recommended in areas where continued oozing is anticipated since meshing prevents development of underlying haematoma. Autografting is not possible in burns involving >40% total body surface area without reusing the donor site again in the next 7-10 days. Chemicals burns cause extensive skin damage. Unlike scalds, chemical burns that appear superficial at presentation soon progress to full-thickness (deep dermal).

In coarctation of the aorta: A congestive failure is usually due to hypertension

(False)

B rupture of the aorta is a recognised complication

(True)

C cerebrovascular haemorrhage is a known hazard

(True)

D infective endocarditis of the bicuspid valves is a very rare occurence

(False)

E Associated hypertension is most frequently due to isolated systolic hypertension.

(False)

Comments: a-Usually secondary to valvular disease. Aortic rupture may occur due to associated medial disease and stroke as a consequnece of hypertension is also a feature. Bicuspid valvular disease is more frequently associated with calcification but prophylaxis to SBE should be offered in reference to certain procedures due to the risk of infective endocarditis. Combined diastolic and systolic hypertension is typical.

The following statements on Down's syndrome are correct: A the overall risk of having a child with Down's syndrome, irrespective of maternal age, is 1 in 650 live births.

(True)

B the risk of having another baby with trisomy 21 after the birth of a child with Down's syndrome is the same as that seen in the general population.

(False)

C Down's syndrome is due to a 14;21 translocation in 30% of cases.

(False)

D hyperthyroidism is commonly found in children with Down's syndrome.

(False)

E 30% of children with Down's syndrome have congenital cardiac defects.

(True)

Comments: The risk of having a child with Down's syndrome increases with maternal age, reaching 1 in 40 in women over the age of 44 years. After having had one child with Down's syndrome the risk of having another child with trisomy 21 is 1 in 200. In about 5% of cases, Down's syndrome

Pediatrics109

can be shown to be due to a translocation. In less than half these cases one of the parents can be shown to be carrying a balanced version of the translocation. Hypothyroidism is commonly seen in children with Down's syndrome and thyroid function tests should be performed annually. From Hannam et al. MRCP (Paediatrics) Part 1 MCQs. page 77 © WB Saunders. Reproduced with permission.

Physiological jaundice in the full term newborn infant is associated with: A Urobilinogen in the urine.

(False)

B Large doses of Vitamin K.

(False)

C Pregnanediol in breast milk.

(False)

D Reticulocytosis.

(False)

E Itching in the infant.

(False)

Comments: Physiological jaundice in the newborn occurs in 90% of infants and is a result of increased rate of red cell breakdown and hence bilirubin accumulation secondary to impaired hepatic clearance.

A baby girl is considered normal at birth but on the second day of age cyanosis is noted. No murmur is heard and there is no respiratory distress. On the third day, cyanosis is more obvious and the respiratory rate is increased. Which of the following is correct concerning this patient? A Cyanosis is most likely due to congenital heart defect

(True)

B The baby’s colour should improve rapidly after giving 100% 02

(False)

C Echocardiography can often tell the anatomic state of the newborn’s circulation

(True)

D The baby’s chest x-ray shows a small heart. This is against the diagnosis of congenital heart disease.

(False)

E The lack of murmur is against the diagnosis of CHD.

(False)

Comments: Cyanosis is most likely due to a congenital heart defect and a right to left shunt. 100% Oxygen will not improve the degree of cyanosis in the presence of a right to left shunt where deoxygenated blood is entering the systemic circulation. Echocardiogram has a high diagnostic accuracy rate and increasingly surgery is performed following echo findings without interim catheterisation. Certain congenital heart defects such as tetralogy of fallot are associated with small cardiac size. Cyanosis is often the only clinical sign present in patients with CHD.

Concerning the development of the cardiovascular system:

Pediatrics110

A the main pulmonary artery develops from the left 6th branchial arch.

(True)

B a right-sided aortic arch is usually present at 10 weeks.

(True)

C the ductus arteriosus is the dorsal remnant of the right sided aortic arch.

(True)

D the septum secundum grows up as a limbus of the foramen ovale.

(True)

E atrioventricular septal defects (AVSD) may involve defects of the septum primum.

(True)

Comments: The blood and cardiovascular system are derived form mesoderm and first appear in the middle of the 3rd week. Bilateral ventral tubes fuse and by 21 days there is a single ventral heart tube that begins to beat by about the 23rd day. By 28 days the definitive chambers of the heart are apparent. In the 5th week, septal ridges grow together to septate the heart. The septum primum appears as a crescent-shaped downward growth of the postero-superior wall of the common atrium. Before the septum primum is complete, small openings appear in the upper portion; these merge to form the ostium secundum. On the right of the septum primum the thicker septum secundum grows down. It becomes complete except for a defect that becomes the oval foramen. From Hannam et al. MRCP (Paediatrics) Part 1 MCQs. page 45 © WB Saunders. Reproduced with permission.

Which of the following are true of osteomyelitis? A Is most commonly due to a staphylococcus aureus infection

(True)

B Can be due to salmonella infection in patients with sickle cell anaemia

(True)

C Infection usually involves the metaphysis of long bones

(True)

D Dead bone within the medullary canal is known as the involucrum

(False)

E New bone forming beneath the periosteum is known a the sequestrum

(False)

Comments: Osteomyelitis is usually due to staphylococcus aureus infection. Streptococcus pyogenes, Haemophilis influenza and gram-negative organisms can also infect bone. In children the infection is usually aquired by haematogenous spread to the metaphysis of long bones. The dead bone within the medullary canal is the sequestrum. New subperiosteal bone formation is the involucrum.

In vascular endothelium: A nitric oxide (NO) is produced from citrulline by the action of enzyme nitric oxide synthatase (NOS).

(False)

B endothelial derived relaxing factor (EDRF) is the molecule NO.

(True)

C NO produces vasorelaxation by increasing cGMP production.

(True)

D released endogenous NO has a half-life of about 20 seconds.

(False)

Pediatrics111

E tachyphylaxis in the pulmonary circulation commonly occurs when NO is used as an inhaled therapy.

(False)

Comments: Arginine under the action of NOS gives rise to NO and citrulline. EDRF has been shown to be the molecule NO, which has a free radical structure with an extra electron. For this reason it is short lived with a half-life of about 6-7 seconds. Tachyphylaxis has rarely been reported. From Hannam et al. MRCP (Paediatrics) Part 1 MCQs. page 131 © WB Saunders. Reproduced with permission.

Increased gastric emptying that may alter the rate of drug absorption is found in the following conditions: A coeliac disease.

(True)

B raised intracranial pressure.

(False)

C migraine.

(False)

D duodenal ulcer.

(True)

E gastric ulcer.

(False)

Comments: Gastric emptying is an important determinant of rate and sometimes extent of drug absorption. Increased gastric emptying is associated with coeliac disease, duodenal ulcer and gastroenterostomy. Decreased gastric emptying occurs with raised intracranial pressure, migraine, pyloric stenosis, trauma, severe pain, gastric ulcer and intestinal obstruction. From Hannam et al. MRCP (Paediatrics) Part 1 MCQs. page 51 © WB Saunders. Reproduced with permission.

Immunoglobulin G (IgG): A crosses the placenta.

(True)

B is secreted in breast milk in significant quantities.

(False)

C levels decrease in preterm neonates after exchange transfusion.

(False)

D levels are lowest 4-6 months post-term.

(True)

E provides immunity to tuberculosis.

(False)

Comments: Immunoglobulin G (IgG)crosses the placenta from as early as 12 weeks gestation. This is initially a slow process and transfer increases rapidly only after about 30 weeks gestation. Postnatal levels of IgG decline, reaching a nadir at approximately 4-6 months, until endogenous production becomes significant. The antibody content of colostrum and breast milk is almost entirely immunoglobulin A. Cell-mediated mechanisms provide immunity to tuberculosis. From Hannam et al. MRCP (Paediatrics) Part 1 MCQs. page 15 © WB Saunders. Reproduced with permission

Pediatrics112

The presence of the following are characteristic features of a large ventricular septal defect: A Heart failure.

(True)

B Diminished second heart sound.

(False)

C Mid-diastolic murmur at the apex.

(True)

D A plethoric lung field on the CXR.

(True)

E Repeated chest infection.

(True)

Comments: Heart failure, pulmonary plethora and mid diastolic murmur due to increased flow across the mitral valve imply significant shunting. Repeated chest infections are a manifestation of cardiac failure. A large shunt will case pulmonary hypertension and a loud second heart sound. A three month old infant presents with acute dyspnoea and cyanosis. On examination the pulse rate is 180 per minute, he has a grade ¾ pan systolic murmur at the left sternal edge, basal crepitations and a liver measuring 4cm below the right costal margin. A The most likely diagnosis is a ventricular septal defect

(False)

B He requires a diuretic

(True)

C He should be digitalised with 50ug/kg of Digoxin given over 24 hours

(True)

D He should make a spontaneous recovery

(False)

E Chest x-ray would be likely to show a small cardiac shadow

(False)

Comments: Ventricular septal defects cause shunting of oxygenated blood from the left ventricle to the right. Cyanosis is a later occurrence-following the development of Eisenmenger’s syndrome ie shunt reversal. Diuretics are required to offload pulmonary venous congestion. Digoxin has a positive inotropic effect. Cyanotic congenital heart disease requires surgical correction of vascular or shunt anomalies. The differential diagnosis of cyanosis and congestive cardiac failure in neonates includes transposition of great vessels, total anomalous pulmonary venous drainage, hypoplastic left heart , single ventricle and tricuspid valve abnormalities and each is associated with cardiomegaly.

The following are true for errors of morphogenesis (congenital abnormalities): A Teratogens can cause abnormalities by interfering with organogenesis.

(True)

B Malformations and deformations are synonymous.

(False)

C 30% of babies born to mothers with insulin dependent diabetes mellitus present at birth with multiple congenital abnormalities.

(False)

D Single gene defects account for the majority of congenital heart defects.

(False)

Pediatrics113

E In the newborn with multiple congenital abnormalities chromosomal aberrations are always found.

(False)

Comments: Malformations include any structural, functional or biochemical abnormality determined genetically or induced during gestation and not due to birthing events. Deformation is a specific developmental anomaly of one organ or tissue. The incidence of birth defects is approximately 1%. Teratogens, maternal infections such as rubella may be the cause of multiple congenital abnormalities without any chromosomal abnormalities.

The following statements are correct: A following the introduction of the MMR vaccine it is no longer necessary to vaccinate against rubella in 10 to 14-year-old girls.

(False)

B the Hib vaccine is a capsular conjugated polysaccharide.

(True)

C immunoglobulin must not be given with the MMR vaccine.

(True)

D hepatitis B vaccine is contraindicated in pregnancy.

(False)

E the mumps component of the MMR is a live vaccine.

(True)

Comments: From September 1994 the MR vaccine should be given to all children aged 5-16 years in school. There are two brands of Hib vaccines, both of which are capsular conjugated polysaccharide vaccines. They are not interchangeable since they are conjugated with different proteins. Immunoglobulin must not be given with the MMR since the immune response to rubella and mumps may be inhibited. Live vaccines are not routinely given to pregnant women but if tehre is a significant risk of exposure the benefits of vaccination may outweigh the risks to the fetus. Since the hepatitis B vaccine contains inactivated virus surface antigen (HBsAg) it can be given in pregnancy. All parts of the MMR are live. From Hannam et al. MRCP (Paediatrics) Part 1 MCQs. page 83 © WB Saunders. Reproduced with permission. The following statements are correct: A it is recommended that folate supplements should be taken by all pregnant women in order to prevent neural tube defects.

(True)

B the dose of folate necessary to prevent neural tube defects in a first pregnancy is 5 mg.

(False)

C pregnant women who are taking phenytoin may need to increase their dose of folate.

(True)

D the recommended dose of folate in a pregnant woman who has had a previous baby with a neural tube defect is 5 mg.

(True)

E supplementation with folate should continue throughout pregnancy.

(False)

Comments: It has been proven that supplementing the diet with folate can prevent neural tube defects. Prevention in women in their first pregnancy should start in the preconception period. A dose of 400 mcg is recommended and should be continued until the 12th week of pregnancy. In a

Pediatrics114

woman who has previously had a baby with a neural tube defect a higher dose of 5mg is recommended. Folate levels can be reduced in women on phenytoin. For this reason pregnant women on this medication should have an increased dose of folate. From Hannam et al. MRCP (Paediatrics) Part 1 MCQs. page 15 © WB Saunders. Reproduced with permission.

Cyanosis in infancy is a common feature of A Ventricular septal defect

(False)

B Transposition of the great vessels

(True)

C Atrial septal defect

(False)

D Coarctation of the aorta

(False)

E Tricuspid atresia

(True)

Comments: Cyanosis is not a feature of VSD unless associated with Eisenmenger’s syndrome. Transposition of the great arteries, Fallot’s tetralogy, tricuspid atresia and single ventricle are associated with cyanosis. ASD is associated with increased pulmonary flow. Coarctation causes obstruction to outflow with ventricular hypertrophy and hypertension (usually in the upper limbs) with radio-femoral delay.

Regarding Ewing’s sarcoma: A Commonly arises towards the end of long bones in the metaphysis

(False)

B Is more common in children

(True)

C Associated with a t11:22 chromosomal translocation

(True)

D X-rays show bone destruction and new bone formation, often with marked periosteal elevation

(False)

E Could arise from long-standing Paget’s disease

(False)

Comments: Ewing’s sarcoma is a malignant round-cell tumour that occurs in the diaphysis of long bones (Osteosarcoma affects the metaphysis) and limb girdles, usually in children. The x-ray appearance described in the choice of answers is a feature of osteosarcoma; in Ewing’s sarcoma, x-rays often show a large soft-tissue mass with concentric layers of new bone formation – known as ‘onion-peel’ sign. Paget’s disease could lead to Osteosarcoma in approximately 10% of patients affected for >10 years, but not to Ewing’s sarcoma.

In childhood offensive loose stools may be caused by: A increased secretions of pancreatic enzymes.

(False)

B Bodian-Schwachmann syndrome.

(True)

C biliary atresia.

(True)

Pediatrics115

D cystic fibrosis.

(True)

E abetalipoproteinaemia.

(True)

Comments: A decrease or absence of pancreatic enzyme secretion may cause loose, offensive stools. Chest infections or respiratory signs are suggestive of cystic fibrosis. Bodian-Schwachmann syndrome presents with failure to thrive in the absence of respiratory symptoms, persistent neutropenia, thrombocytopenia, raised fetal haemoglobin and minor liver enzyme abnormalities. Growth retardation, skeletal dysostoses and bone marrow hypoplasia may also be present. Abetalipoproteinaemia results from a defective production of apoprotein B by the intestinal cells leading to defective synthesis of low-density lipoproteins, very low-density lipoproteins and chylomicrons (CLM). Absence of CLM results in fat malabsorption, deficiency of fat-soluble vitamins and steatorrhoea. Very low cholesterol (