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1-A 7-year-old boy presents with tall stature.

On investigation, his bone age


is accelerated by one year.
The following should be considered:
True / False
Marfan's syndrome
Hyperthyroidism

Correct
Correct

Congenital adrenal hyperplasia


Beckwith-Wiedemann syndrome
Precocious puberty

Correct
Correct

Correct

The majority of tall children have a familial determinant for it. Other causes
include:
Hormonal

excess adrenal androgens

excess sex steroids

excess pituitary growth hormone (very rare)

hyperthyroidism.
Syndromes

Marfan's

homocystinuria

Klinefelter's

Sotos

Beckwith-Wiedemann syndrome.

In this case it would be unusual for congenital adrenal hyperplasia to present


so late, but in boys who are not salt losers, a late presentation is possible.
Beckwith-Wiedemann syndrome can cause tall stature, but presents in the
neonatal period, not at seven years.
The accelerated bone age suggests a hormonal rather than syndromic cause.

2-A child is noticed to have palpable purpura on the elbows. Which one of
the following is most compatible with a diagnosis of Henoch-Schonlein
syndrome?
(Please select 1 option)
Haemolytic anaemia
Petechiae

Correct

Splenomegaly
Sudden onset of oedema in the hands and scrotum
watery diarrhoea

Thrombocytopenia , haemolysis and splenomegaly are not present and


clotting is normal. Subcutaneous oedema of the feet, hands, scalp and ears
are seen. Scrotal oedema also may occur. Gastrointestinal bleeding may
occur leading to bloody stools.
Haematuria and proteinuria also may occur. Abdominal pain, intussusception
and arthritis are features. Petechiae, purpura and papules are commonly
present in the thighs and buttocks.
Next question

3-For a group of young men with height 160-190cm the PEFR was
measured. The results were plotted on a scatter diagram and a linear
regression line drawn using the least squares method. Which of the following
statements are correct?
True / False
the correlation coefficient can't be higher than 1

Correct

if the correlation coefficient is 0 there is no linear relationship between height and


PEFR

Correct

if the correlation coefficient is positive, the curve would have an upward slope
Incorrect answer selected
this data can be used to calculate the PEFR for a height of 150cm
height should be on the vertical axis on the graph

Correct

Correct

a- -1 to +1 d- if a correlation can be made then figures can be extrapolated and


150cm isn't too far from 160cm e-PEFR is the dependent variable and is usually put
on the Y (vertical) axis. (height is fixed and is on the X (horizontal axis).

4-Which of the following immunisations are recommended in a child aged between 14-16
years?
(Please select 1 option)
BCG
Haemophilus influenzae B
MMR
Pertussis

Tetanus

Correct

A booster tetanus and diphtheria vaccination is required at aged 14-16 years.


These boosters are recommended every 10 years. A second dose of MMR is
given in some countries at age 12. BCG vaccination is offered to various
groups according to risk and is not therefore routine.
Further reading: NHS Green Book
Next question

5-Theme:Cutaneous manifestations of infectious disease


A

Rheumatic fever

TB

Lyme disease

Chicken pox

Histoplasmosis

Cat scratch disease

Measles

Parvovirus

Hepatitis B

Herpes simplex

For each cutaneous manifestation described below choose the single most
likely associated infectious disease.
Erythema nodosum in presence of abnormal chest x ray.

Correct

Erythema nodosum may be associated with streptococcal reactions, rheumatic fever


and tuberculosis. In the presence of an abnormal chest x ray TB is the most likely
answer.
Erythema marginatum.

Correct

Erythema marginatum is one of the five major criteria to make a diagnosis of


rheumatic fever. It is a pink rash with pale centres and a serpiginous margin, found
on the trunk and proximal limbs.
Erythema chronicum migrans.

Correct

Erythema chronicum migrans found in Lyme disease. A febrile illness caused


by Borrelia burgdorferi transmitted by bites of animal tick. The rash is characterised
by red margins and central clearing.
Next question

6-A 17-year-old female presents with tingling and muscle cramps. There is
no other past medical history of note.
Investigations reveal:
Creatinine

68 mol/L (60-110)

Calcium

1.76 mmol/L (2.2-2.6)

Albumin

38 g/L (37-49)

Which one of the following investigations is most likely to confirm the


diagnosis?
(Please select 1 option)
Alkaline phosphatase concentration
CT brain scanning
PTH concentration

Correct

Urine calcium concentration


Vitamin D concentration

This patient has low calcium which could be due to either vitamin D
deficiency or hypoparathyroidism.
The most likely cause in a young patient who has otherwise been quite well
with normal renal function would therefore be hypoparathyroidism.
Urine calcium concentrations are useful in familial hypercalciuric
hypercalcaemia.
Next question

7-15 month old child is playing alone while mother is preparing her supper.
Suddenly mother hears a sharp coughing from the playroom, and finds her
daughter coughing and choking, with a red face and lacrimation. She slaps
her back and calls an ambulance. The ambulance crew give oxygen.
On arrival she has O2 saturations of 97% in air. Her respiratory rate is 20/
minute, and there is no recession.
What is the most likely diagnosis?
(Please select 1 option)
Asthma
Factitious and induced illness
Gastroesophageal reflux and aspiration
Inhalation of foreign body

Correct

Pertussis

The history is suggestive of aspiration of a small object. Often there is acute


coughing, then symptoms settle. It is important to do a chest X-ray to identify any
radio-opaque object. Remember that non-opaque objects will not show up, but that
secondary inflammation may follow, resulting in chronic problems eg right middle
lobe bronchiectasis.
Next question

8-The following vaccines contain live virus:


True / False
Rubella
Influenza
Mumps

Correct
Correct
Correct

Rabies

Correct

Hepatitis B

Correct

Rubella and Mumps vaccines contain live virus. Influenza is inactivated, either split
virion or surface antigen. The rabies virus contains killed virus and the Hepatitis B
vaccine contains purified viral proteins.
Next question

Rate this question

9-Theme:Childhood Hypertension.
A

DMSA scan

Echocardiogram

Plasma renin and aldosterone

Renal angiography

Thyroid function tests

Urine catecholamines

Urine drug screen

U+Es, creatinine

I
Ultarsound KUB
For each scenario choose them most specific investigation:
A 15-year-old girl is brought to A+E having collapsed at a party. On examination she has a dry
mouth, a heart rate of 110/min and blood pressure of 150/110 mmHg.

Correct

In the 15-year-old girl there is acute hypertension, most likely related to drugs. The
most common offender these days is probably MDMA (ecstasy). A drug screen will
confirm this.
A 6-year-old boy presents with headache on defaecation. On examination his blood pressure in
the right arm is 140/100 mmHg and he has a 2/6 systolic murmur.

Correct

The 6-year-old boy has hypertension and a heart murmur. It is important to exclude
coarctation. The murmur for this may be loudest over the scapula. Four limb BPs are
usually diagnostic, but echocardiography is will identify the precise structural lesion.

A 2 month old infant is found to have a blood pressure of 120/90 mmHg persistently. He was
born at 25/40 gestation, was ventilated for 14 days and is still in oxygen.

Incorrect - The correct answer is Renal angiography

The 2 month old infant is likely to have renal artery thrombosis complicating UAC
insertion. Renal angiography will confirm this.
In evaluating raised blood pressure first decide whether it is acute or chronic. Second
is it related to drugs. Third is it localised.
Next question

10-Primary hypothyroidism is associated with the following in childhood:


True / False
Macrocytic anaemia
Alopecia areata
Cerebellar ataxia

Incorrect answer selected

Correct
Correct

Multiple serous effusions


Addison's disease

Correct

Correct

Hypothyroidism has the following clinical features:

INFANTS: Feeding problems, prolonged jaundice, constipation, pale


cold skin, coarse facies, large tongue, hoarse cry, occasional goitre,
umbilical hernia, delayed development. Oedema of the genitalia and
pericaridal effusion can occur.

CHILDREN: Cold intolerance, dry skin, cold peripheries, bradycardia,


dry thin hair, pale puffy eyes with loss of eyebrows, goitre, slow relaxing
reflexes, constipation, short stature, delayed puberty, obesity,
deteriorating school work, learning difficulties, hyperprolactanaemia.
Macrocytic anaemia is often present.

There is a known association with autoimmune diseases such as alopecia,


vitiligo, pernicious anaemia, Addison's Disease.
Copyright 2002 Dr Colin Melville
Next question

11-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:
True / False
A. Buccal mucosa chromatin -ve

Correct

B. High urinary 17-ketosteroid excretion


C. Pubic hair present

Correct

Incorrect answer selected

D. Abnormally tall stature


E. Retarded bone maturation

Incorrect answer selected


Correct

A. Females have a Barr body.


B., C., D. These suggest CAH and virilisation.
E. Not a feature at this age, would expect advanced bone age in a female.
Next question

12-An obese 14-year-old boy presents acutely with hip pain, which began
the previous day and has prevented walking. He has previously been well.
Full term normal delivery, no neonatal problems. Immunisations up to date.
No family or social history of note.
On examination the temperature is 36.5C, RR 12/min and HR 80/min. He
has severe limitation of movements of the right hip because of the degree of
discomfort. The hip is held flexed and externally rotated.
What is the most likely diagnosis?
(Please select 1 option)
Legg-Calve-Perthe
Osteomyelitis
Reactive arthritis
Septic arthritis
Slipped capital femoral epiphysis

Correct

The history of acute hip pain in an obese adolescent without fever suggests a slipped
capital femoral epiphysis (SCFE). It may present subacutely or with pain referred to

13-the knee. In the subacute case the child walks painfully with the knee externally
rotated on the affected side. The SCFE is pinned to prevent further slippage.
Next question

A 6 month old girl presents with unresponsiveness. She had a slight cold the
night before, but when mother went to wake her this morning she was
drowsy and lethargic. She also had a purple rash over her arms. She was
born at 36+4/40 3.1kg and there were no neonatal complications. She had
been thriving.
Her temperature was 35.9C(tympanic), RR 35/min and HR 150/min. She
has cool peripheries and a capillary refill time of 4 seconds. She has a nonblanching rash over her arms, trunk and legs.
What is the most likely diagnosis?
(Please select 1 option)
Diabetic ketoacidosis
Hepatic failure
Hypoglycaemia
Infection eg meningitis, encephalitis

Correct

Substance abuse

She has purpura fulminans. This is meningococcal septicaemia until proved


otherwise. She requires rapid resuscitation, including securing the airway and
breathing and fluid boluses till she is out of shock. Cefotaxime or ceftriaxone
will cover the common organisms
(menincococcus, pneumococcus and Haemophilus influenzae).
Finish

14A 17-year-old man has been diagnosed with schizophrenia 4 weeks ago. He was started on
haloperidol. Two weeks later he was found confused and drowsy. On examination he was
pyrexial (40.7oC), rigid with blood pressure of 200/100. Which of the following treatment will
you initiate?
(Please select 1 option)
phenytoin
diazepam
cefuroxime
acyclovir

dantrolene

Correct

Neuroleptic malignant syndrome is the most likely diagnosis. Its major features are:
rigidity, altered mental state, autonomic dysfunction, fever, and high creatinine
kinase. It is usually caused by potent neuroleptics. The treatment of choice is
dantrolene and bromocriptine. Withdrawal of neuroleptic treatment is mandatory.
Rhabdomyolysis and acute renal failure are potential complications.
Next question

15-The following statements about hepatitis are true:


True / False
Hepatitis C rarely leads to a chronic condition.

Correct

A raised IgM antibody titre confirms the diagnosis of hepatitis A.

Incorrect

answer selected
Hepatitis A vaccine is very effective.

Incorrect answer selected

Hbs AB persists for more than 10 years.

Correct

Hbe Ag is associated with increased infectivity.

Correct

Hepatitis C is a transfusion-related virus that is causing immense problems to


the Blood Transfusion Service. Hepatitis A and E cause acute disease, but B,
C and D can all cause chronic hepatitis.
Copyright 2002 Dr Colin Melville
Next question

16-A 7-year-old girl presents with crampy lower abdominal pain, loose stools
and anorexia of 3 days duration. The stools contain blood and slime and
occur 20 times a day. There is no history of foreign travel. She was born at
38+4/40 weighing 3.2kg and there were no neonatal problems.
On examination she appears lethargic and pale. She has dry mucous
membranes but no jaundice. Her temperature is 37.6C (tympanic), HR
100/min, RR 15/min. She has a few purpuric spots on her legs.
What is the most likely diagnosis?
(Please select 1 option)
Marrow failure

Incorrect answer selected

Haemaglobinopathies eg Sickle, Thalassaemia


Haemolytic uraemic syndrome

This is the correct answer

Iron deficiency
Malignancy

The history suggests a dysenteric illness followed by haemolytic uraemic


syndrome. This is now the commonest cause of acquired acute renal failure
in childhood. The likely organism is Escherichia coli0157:H7 in the UK,
although Shigella, Campylobacter and a variety of viruses have also been
described. These trigger the triad of microangiopathic haemolytic anaemia,
thrombocytopaenia and acute renal failure. A FBC showed Hb 6.8 g/dl, WC
16.8 x 10 , Platelets of 36. Blood film showed schistocytes, spherocytes,
helmet cells and polychromasia. Careful attention to fluid balance is essential
to avoid fluid and electrolyte-related complications.
9

Next question

17-Neuroblastoma
True / False
is an embryonic tumour

Correct

usually has a familial incidence

Correct

metastasises most commonly to the lungs


is easily eradicated by radiotherapy
is a secreting tumour

Incorrect answer selected

Correct

Correct

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 recognised in this condition. Extra abdominal sites
generally have a better prognosis. It metastasises most commonly to lymph nodes,
bone, bone marrow, liver, and skin.
Next question

18-Theme:Childhood Diarrhoea
A

Adenovirus

Cytomegalovirus

Epstein-Barr virus

Escherichia coli

Giardia lamblia

Hepatitis A

Norwalk virus

Rotavirus

Salmonella species

J Staphylococcus aureus
Select one option from the list above that is most suitable for the following patients
Accepted as the most common cause of infectious diarrhoea in children in the developed
society.

Correct

Rotavirus is the most common virus responsible for diarrhoea worldwide. It is a


double stranded RNA virus, especially prevalent in the winter months. It causes fever
and watery diarrhoea.
Can follow ingestion of dust containing dried faecal material.

Correct

Hepatitis A is an RNA virus typically transmitted by the faecal-oral route.


Attaches the small intestinal border and releases exotoxin.

Correct

E-coli is an enteropathogen and one of the bacterial causes of diarrhoea. Many of its
effects are a consequence of an exotoxin. It should be suspected if there is a history
of travel or a history of ingestion of poorly prepared food. Bacterial diarrhoea usually
results in bloody diarrhoea.
Next question

19-An 16-year-old female is found to be hypertensive at 26 weeks of her


first pregnancy.
Blood pressure

144/92 mmHg

Dipstick urine

+++ proteinuria

Serum creatinine

80 mol/L

24 hour urine collection

1.2 g of protein/d

What is the most appropriate management?


(Please select 1 option)

Atenolol
Methyldopa

Correct

Nifedipine
Ramipril
Re-measure blood pressure in one week

This patient has Pre-eclampsia, which is pregnancy induced hypertension with


proteinuria with or without oedema; a multisystem disorder originating in the
placenta. As BP is >140/90 with associated proteinuria she needs admission for
observation and monitoring of BP, biochemistry, daily weight, fluid balance and
monitoring of urinary protein. Methyldopa would be the agent of choice to control
hypertension. ACEi are contraindicated in pregnancy due to teratogenicity.
Next question

20-A 4-month-old boy presents with heart murmur, breathlessness and


failure to thrive.
He has always breathed a little fast, but this has gradually worsened. He has
been falling through the centiles. He was born at 40+2/40 weighing 3.22 kg
and there were no neonatal problems. Immunisations are up to date. There
is no family or social history of note.
On examination the temperature is 36.9C, respiratory rate 40/min (minimal
recession) and pulse is 120/min. He is below the 3% for weight and on the
50% for head circumference.
He has an active praecordium, loud P2 and a 3/6 pansystolic murmur
maximal at the lower left sternal edge, but heard all over the praecordium.
The liver is 3 cm.
What is the most likely diagnosis?
(Please select 1 option)
Atrial septal defect, primum
Atrial septal defect, secundum
Mitral regurgitation
Tricuspid atresia
Ventricular septal defect (VSD)

Correct

This history suggests heart failure, worsening over the first few weeks of life,
with a pansystolic murmur.
This suggests a VSD causing haemodynamically significant left-to-right
shunt. The shunt volume usually increases in the first few weeks as the
pulmonary vascular resistance drops. The murmur goes from ejection systolic
in the first few days, gradually becoming pansystolic. Diuretics are often
needed.
Some VSDs (particularly of the muscular septum) close spontaneously.
Others (especially of the membranous septum) require surgical closure.
Antibiotic prophylaxis is essential to reduce the risk of endocarditis.
Next question

21-A 3-year-old girl presents with pallor, lethargy and bruising.


She has been unwell for about three weeks, but seems to have deteriorated
over the past few days. She was born at 40+2/40 gestation weighing 3.0 kg
and there were no neonatal problems.
On examination she appears very pale, and has petechiae over the palate
and arms. She looks unwell. She is on the 25 centile for height and weight
and has no dysmorphic features.
th

What is the most likely diagnosis?


(Please select 1 option)
Haemaglobinopathies e.g. sickle, thalassaemia
Iron deficiency
Malignancy

Incorrect answer selected

Marrow failure

This is the correct answer

Occult blood loss

The history suggests bone marrow failure of subacute onset, with at least
two cell lines involved (red cells and platelets).
The presence of hepatosplenomegaly or lymphadenopathy might suggest
acute leukaemia, but in their absence aplastic anaemia is a strong possibility.
This can be confirmed by bone marrow aspirate, which shows marrow
hypoplasia.

Most are idiopathic.


Underlying congenital causes are present 20%, and include

Fanconi anaemia

Diamond-Blackfan

cartilage hair hypoplasia

Pearson syndrome.

Underlying acquired causes include

infections (Epstein-Barr virus [EBV], parvovirus, human


immunodeficiency virus [HIV])

toxins and drugs

pregnancy

transplant-related.
Next question

22-A 17-year-old female presents with a severe headache of sudden onset


associated with nausea and photophobia.
On examination there was evidence of meningism. She had a previous
history of migraine without aura and was taking the oral contraceptive pill.
Which of the following is the most appropriate initial investigation?
(Please select 1 option)
Cervical spine x ray
CT scan of head

Correct

Lumbar puncture
MRI scan of head
Skull x ray

This presentation suggests the diagnosis of subarachnoid haemorrhage.


The first line investigation is a computerised tomography (CT) head scan
looking for evidence of haemorrhage.

If this is not demonstrated it should be followed by a lumbar puncture as


subarachnoid blood may not be evident in up to 10% of cases on CT scan .
Next question

23-A 16-year-old female attends casualty 15 hours after ingesting


approximately 30 g of paracetamol and 2 g of dihydrocodeine.
On examination, she is drowsy with a Glasgow coma scale of 15. Her pulse is
100 beats per minute, blood pressure is 110/66 mmHg and she has pinpoint
pupils, with saturations of 96% on air.
What is the most appropriate treatment for this patient?
(Please select 1 option)
10% dextrose infusion
Activated charcoal by mouth
Gastric lavage
N-acetylcysteine intravenously

Correct

Naloxone intravenously

This patient has taken a significant overdose of paracetamol, and is


presenting late at 15 hours.
She is at risk of hepatocellular damage and needs to be commenced on an
infusion of intravenous N-acetylcysteine immediately. It is continued for 30
hours and sometimes beyond this, depending on results of prothrombin time,
liver function tests, urea and electrolytes, and glucose.
Activated charcoal is given to patients presenting within one hour of
overdose.
Flumazenil is reserved for reversal of benzodiazepine-induced respiratory
depression.
Naloxone is used for opiate-induced respiratory depression.
Next question

24-Intraosseous cannulation for the infusion of fluids and drugs


(Please select 1 option)

Is a technique frequently performed on paediatric and adult patients


Should be considered when attempts at gaining peripheral venous access are prolonged
Correct
In the presence of limb bone fractures, the intraosseous cannula should be inserted at the
fracture site
Is rarely indicated during a paediatric cardiac arrest
In the tibia an intraosseous cannula should be inserted in the midline at the level of the tibial
tuberosity

First used in the 1930s as an alternative method of gaining vascular access,


intraosseous cannulation is currently recommended during life threatening
situations when vascular access is required quickly. Therefore, when
attempts at gaining venous access fail or are delayed, intraosseous
cannulation should be considered.
In paediatric cardiac arrest it is the recommended technique for gaining
circulatory access.
Many potential anatomical sites for intraosseous cannulation have been
described, including the lower and upper limbs and sternum.
The correct site on the proximal tibia is 2 to 3 cm below the tibial tuberosity
on the antero-medial surface. Fracture sites should be avoided, as should
limbs with proximal fractures.
Next question

25-An 18-year-old man had repeated episodes of breathlessness and


palpitations, lasting about 20 minutes and resolving gradually. There were no
abnormal physical signs.
What is the most likely cause of these features?
(Please select 1 option)
Amphetamine abuse

Incorrect answer selected

Generalised anxiety disorder


Panic disorder

This is the correct answer

Social anxiety disorder


Thyrotoxicosis

Drug abuse is unlikely since the symptoms are quite shortlived. We would
expect other symptoms such as gastrointestinal disturbance, headaches or
hypertension to accompany a variety of drug abuse causes.
Thyrotoxicosis would be expected to lead to symptoms of longer duration
with other associated symptoms.
Panic disorder is the most likely diagnosis.
Next question

26-A 16-year-old girl presented with fever, headache and photophobia.


Investigations revealed:
Cerebrospinal fluid examination
Opening pressure

260 mm H20

(50-180)

Total protein

0.8 g/l

(0.15-0.45)

Glucose

4.2 mmol/l

(3.3-4.4)

White cell count

60 per ml

(<5)

Lymphocytes

90%

(60-70)

Plasma glucose

6.4 mmol/l

(3.0-6.0)

What is the most likely diagnosis?


(Please select 1 option)
Bacterial meningitis
Cryptococcal meningitis
Tuberculosis meningitis

Incorrect answer selected

Viral encephalitis
Viral meningitis

This is the correct answer

A normal cerbrospinal fluid (CSF) glucose together with

a CSF lymphocytosis

an increased opening pressure

a raised CSF protein

are typical of a viral meningitis, which would be high on the list of


differentials in patients of this age group (together with bacterial meningitis).
Further reading:
Cerebrospinal Fluid Analysis American Academy of Family Physicians
Next question

27-Concerning neurofibromatosis type 1 (NF1), which one of the following


statements is true?
(Please select 1 option)
Bilateral acoustic neuromas are common
Clinical severity in individuals is similar in a given family

Incorrect answer selected

New mutations occur rarely


Pigmented spots on the iris are a characteristic feature

This is the correct answer

The diagnosis is likely if two caf-au-lait patches are present

Lisch nodules of the iris are present in more than 90% of patients.
Bilateral acoustic neuromas are a hallmark feature of neurofibromatosis type
2.
Expressivity of the gene is highly variable and members of the same family
usually show wide differences in clinical symptoms.
NF1 is one of the most common autosomal dominant conditions. However
almost half of all cases give no family history and are new mutations. The
mutation rate is estimated to be 1:10,000 gametes.
The diagnosis is suggested by six or more caf-au-lait macules (spots), each
over 5 mm in diameter in prepubescent individuals and over 15 mm in
postpubertal individuals.
Next question

28-A 17-year-old male is admitted to casualty after a night out with friends
at a nightclub. After drinking eight pints of beer, he felt nauseated and
vomited. Vomitus was seen to contain streaks of bright red blood after
vomiting a second time. His pulse rate is 70/min with warm peripheries, and
he is passing copious quantities of urine.
Investigations revealed:

Haemoglobin

13.2 g/dL (13.0-18.0)

White cell count

10.7 x109/L (4-11 x109)

Platelets

377 x109/L (150-400 x109)

Prothrombin time

12.0 s (11.5-15.5)

Activated Partial Thromboplastin Time

27 secs (30-40)

Blood alcohol

83 mg/dL

What is the next step in his management?


(Please select 1 option)
Coeliac axis angiography
Discharge the patient from hospital

Correct

Prescribe a proton pump inhibitor and admit the patient overnight for observation
Refer to the surgeons for review
Discharge the patient and arrange an endoscopy in one month

The history is compatible with a Mallory-Weiss tear. His investigations are


normal, with a moderate blood alcohol level. No additional treatment or
follow-up is required and the patient can be discharged from hospital.
Next question

29-Microalbuminuria in diabetes mellitus:


True / False
Improves with exercise.

Incorrect answer selected

Can be completely reversed with improved diabetic control.

Incorrect answer

selected
Is a marker of renovascular disease.

Correct

Means the loss in urine of protein with a lower molecular weight than albumin.
Incorrect answer selected
Can be screened for using an early morning spot sample of urine.

Correct

A critical stage in the development of diabetic renal disease is the onset of


microalbuminuria, defined as an albumin excretion rate of 30-300mg per day.
Albumin/Creatinine ratio in a spot urine is a reasonable screening test for it.

Microalbuminuria predicts progression to renal failure and early


cardiovascular mortality in both Type 1 and Type 2 diabetes patients.
Microalbuminuria is associated with constellation of other risk factors for
small and large vessel damage which includes raised blood pressure, poor
glycaemic control, plasma lipid and clotting factor abnormalities, left
ventricular hypertrophy, and insulin resistance. Treatment with ace inhibitors
corrects albuminuria and prevents progression to persistent proteinuria.
Blood glucose control significantly reduces the risk of progression from
normal albuminuria to microalbuminuria. The treatment of microalbuminuria
appears highly cost beneficial and substantially increases life expectancy. All
diabetic patients aged 12-70 years should be screened and the development
of microalbuminuria should alert the physician to set in motion a programme
of assessment, monitoring and correction of all risk factors for renal and
cardiovascular disease.
Copyright 2002 Dr Colin Melville
Next question

30-A 17-year-old girl is admitted with a 2 day history of rigors due to a


urinary tract infection. On examination she appears unwell, has a Body Mass
Index of 31kg/m , a temperature of 39C; examination is otherwise normal.
2

Initial biochemistry revealed:


Potassium

4 mmol/L (3.5-4.9)

Urea

7 mmol/L (2.5-7.5)

Glucose

33 mmol/L (3.0-6.0)

pH

7.3 (7.36-7.44)

Standard bicarbonate 14 mmol/L (20-28)


Base deficit

-10 mmol/L

Urinalysis negative for ketones

Which one of the following is the best initial treatment for her
hyperglycaemia?
(Please select 1 option)
Metformin
Metformin plus Gliclazide
Pioglitazone
Sliding scale IV insulin infusion

Correct

Subcutaneous insulin mixture

This patient has a metabolic acidosis with pH of 7.3 and low bicarbonate
likely due to sepsis. She is likely to be a type 2 diabetic, given the BMI, with
uncontrolled hyperglycaemia but is unlikely to have classical diabetic
ketoacidosis because the urine is negative for ketones.
It is important that her glycaemia is controlled to promote recovery from the
sepsis. This is best achieved with intravenous insulin initially.
Next question

31-Ulcerative colitis (UC) is associated with which of the following?


True / False
Ankylosing spondylitis
Episcleritis
Psoriasis

Incorrect answer selected

Incorrect answer selected


Correct

Renal tubular acidosis

Incorrect answer selected

Sub-total villous atrophy

Correct

UC only affects the colon, except for the rare backwash ileitis.
Thrombocytosis may be a non-specific sign of inflammation.
Extra-intestinal manifestations tend to occur more commonly with ulcerative
colitis than with Crohn's disease.
Growth retardation may be seen in 10-35% of individuals at diagnosis.
Extra-intestinal manifestations that occur with inflammatory bowel disease
(IBD) include

joint

skin

eye

mouth

hepato-biliary involvement.

Among the skin manifestations, erythema nodosum is most common. There


is also a 2-3x increased incidence of UC and Crohn's in patients with
psoriasis.
Glomerulonephritis and a hypercoagulable state are other rare manifestations
that occur in childhood.

Cerebral thromboembolic disease has been described in children with IBD.


Uveitis occurs in about 5% of children with IBD and is usually asymptomatic
and transient.
Copyright 2002 Dr Colin Melville
Next question

32-Recognised features of abetalipoproteinaemia include:


True / False
A high serum cholesterol

Correct

Abnormal red blood cell morphology

Correct

Advanced atherosclerotic vascular disease


Palmar xanthomas

Correct

Correct

Severe mental retardation

Incorrect answer selected

Abetalipoproteinaemia causes neurodegenerative nervous system changes ataxia with retinitis pigmentosa.
IQ is usually normal.
Other clinical manifestations include steatorrhoea and malabsorption, which
may lead to osteopaenia and skeletal fractures.
Radiologically, there is thickening of the mucosal folds of the duodenum and
jejunum.
Acanthocytes are seen in the peripheral blood film.
Inheritance is autosomal recessive.
Next question

33-In Hirschprung's disease:


True / False
Abdominal distension in the newborn period may be the presenting feature
Correct
Diagnosis can be strongly suggested by rectal examination
The affected segment is grossly dilated on barium enema
There is a male:female ratio of 4:1

Correct

Correct
Correct

The right side of the colon is most commonly affected

Correct

Hirschsprung's disease or congenital aganglionic megacolon is caused by an


abnormal innervation of the bowel, beginning in the internal anal sphincter
and extending proximally to involve a variable length of gut.
Hirschsprung's disease is the most common cause of lower intestinal
obstruction in the neonate, with an overall incidence of 1:5,000 live births.
Males are affected more often than females (4:1), and there is an increased
familial incidence in long segment disease.
Hirschsprung's disease may be associated with other congenital defects
including

Down syndrome

Laurence-Moon-Bardet-Biedl syndrome

Waardenburg syndrome

Cardiovascular abnormalities.

Ninety-nine per cent of full-term infants pass meconium within 48 hours of


birth.
Hirschsprung's disease should be suspected in any full-term infant (the
disease is unusual in preterm infants) with delayed passage of stool.
Failure to pass stool leads to dilatation of the proximal bowel and abdominal
distension. As the bowel dilates, intraluminal pressure increases, resulting in
decreased blood flow and a deterioration of the mucosal barrier.
Stasis allows proliferation of bacteria, which may lead to enterocolitis with
associated sepsis and signs of bowel obstruction.
Rectal examination demonstrates normal anal tone and is usually followed by
an explosive discharge of foul-smelling faeces and gas.
Copyright 2002 Dr Colin Melville

34-Theme:Splenomegaly
A Acute leukaemia

Autoimmune haemolytic
disease

Cytomegalovirus infection

D Epstein-Barr virus infection


E

Neoplasia

Histiocytosis

G Red cell enzyme defect


H Red cell membrane defect
I

Toxoplasmosis

For each scenario choose the most likely diagnosis.


A 14-year-old boy presents with a four day history of fever and difficulty in swallowing. On
examination he has a scanty erythematous rash, tonsillar pus, tender cervical nodes and 3 cm
splenomegaly.

Correct

This boy has a mononucleosis-like illness which is most likely due to EBV.
A 9-month-old male presents with pallor. On examination he appears well, has a tinge of
jaundice and a 3 cm spleen.

Incorrect - The correct answer is Red cell membrane defect

The 9 month old has haemolysis, most likely hereditary spherocytosis. In


some parts of the world (e.g Mediterranean) G6PD deficiency may be more
common.
A 3-year-old girl presents with fever, weight loss and irritability.
On examination she has a temperature of 38.4C, neck stiffness, 4 cm hepatomegaly and 5
cm splenomegaly. There are lytic lesions on skull x ray.

Incorrect - The correct answer is Histiocytosis

This girl probably has a malignancy, and the presentation is characteristic of


histiocytosis.
In the differential diagnosis of splenomegaly the first thing is to establish
whether there is an associated infection. If not, then an FBC and film should
be done. This may reveal

underlying haemolysis

atypical lymphocytes

blast cells.

If normal an USS abdomen will be helpful in identifying

congestion

neoplasia

connective tissue

storage disorders.

Next question

35-The following are likely in severe pyloric stenosis:


True / False
Acid urine

Incorrect answer selected

Hypokalaemia

Correct

Hypochloraemia
Metabolic acidosis
Hypercalcaemia

Correct
Correct
Incorrect answer selected

Pyloric stenosis is the most common congenital obstruction of the stomach


and intestines. It occurs in 1:150 males and 1:175 females. It affects first
born males more commonly and has a high concordance rate among
monozygotic twins.
The main presenting complaint is that of projectile vomiting, which typically
starts in the second or third week of life. The vomitus is not bile stained, as
the condition is caused by the hypertrophy and hyperplasia of the antrum of
the stomach.
Babies with this condition are usually hungry and usually suck well.They
often appear wasted and have the appearance of an old man.

Electrolyte abnormalities are common with the patient often showing

hyponatraemia

hypokalaemia

a profound hypochloraemic metabolic alkalosis.

Next question

36-Theme:Acute vomiting
A Hepatitis A titres
B Liver function tests
C

Serum amylase

D Serum glucose
E

Stool culture

Ultrasound abdomen

G Upper GI endoscopy
H Urine culture

Urine metabolic
screen

For each of the following presentations choose the most helpful diagnostic
investigation.
A 4-year-old girl presents with a three day history of vomiting. On examination she has a
temperature of 39.3C and has tenderness in the left loin.

Correct

This girl is associated with renal angle tenderness, pointing towards an upper
pole renal infection. Urine culture will be diagnostic. If the temperature does
not settle rapidly with antibiotics, then an ultrasound scan of kidneys, ureter,
and bladder (KUB) may identify pelvo-ureteral junction (PUJ) obstruction or
renal abscess.

A 6-year-old girl presents with a history of vomiting and bloody diarrhoea. On examination she
has a fever to 38.6C, and has diffuse abdominal tenderness.

Correct

In this girl there are symptoms of dysentery. In acute cases this is usually
due to bacterial gastroenteritis (food poisoning). A stool culture may identify
the offending organism.
A 3-year-old boy presents with abdominal pain and bilious vomiting. On examination he has
deep slow breathing, dry mucous membranes, cool peripheries and a heart rate of 120/min.

Correct

This child has vomiting, dehydration and Kussmaul breathing. This is most
likely to be diabetic ketoacidosis, so a blood glucose will be confirmatory.

Next question

37-Prophylactic antibiotics are mandatory in the following situations:


True / False
For all children at a school where a severe case of meningococcal disease has
occurred

Correct

For all children in a household with a proven case of pertussis

Correct

Indefinitely to all children who have had a proven urinary tract infection
To kissing contacts of a child with Campylobacter

Correct

Correct

Where a child with primum ASD is undergoing dental extraction

Correct

Prophylactic antibiotics should be used sparingly, and in situations where


evidence has shown them to be effective.

In meningococcal disease: to kissing contacts.

In pertussis: to children under the age of 5, or those at high risk


(immunosuppressed, cerebral palsy).

Congenital heart disease: prophylaxis against subacute bacterial


endocarditis is no longer required (NICE 2008).

In urinary tract infection: following a proven urinary tract infection,


prophylaxis should be continued until full investigations have been
completed. For most grades of reflux, prophylaxis will be continued until
the urethral valve matures (usually between 5 and 6 years).

Copyright 2010 Dr Colin Melville


Next question

38-The carotid body chemoreceptors are:


True / False
A Stimulated by a decrease in pO2 of arterial blood|
B Stimulated by a decrease in pH of arterial blood
C Stimulated in pre-diabetic subjects

Incorrect answer selected


Correct

Correct

D Responsible for activating the cardiorespiratory centers


E Have a blood flow of over 50 ml/100 g per minute

Correct

Incorrect answer selected

This is a discriminating question. The carotid body is a chemoreceptor which


detects the partial pressure of blood oxygen as well as, to a lesser degree,
the partial pressure of carbon dioxide. The carotid body is also sensitive to
pH and temperature. The carotid body is said to have the highest blood flow
of any tissue.
Respiratory arrest and circulatory shock dramatically increase chemoreceptor
activity leading to activation of the cardiorespiratory centres.
The chemoreceptors do not respond directly to glucose levels and thus would
not be elevated by pre-diabetic (i.e. impaired fasting glucose and/or impaired
glucose tolerance) glucose elevations.
Next question

39-Haemophilus influenzae type B (Hib) infection:


True / False

Has a peak mortality in the first year of life


Is the main cause of epiglottitis
May cause otitis media

Correct

Correct

Correct

Rarely causes bacterial meningitis in children


Should normally be treated with penicillin

Incorrect answer selected

Correct

Meningococcus is the commonest cause of bacterial meningitis in children


and group B Streptococcus is the most common cause in neonates.
Due to the effect of vaccination, the incidence of new cases of Hib has
dropped from 40-100/100
40-100/100
,000 to 1 per 100,000.
The peak mortality is in the first year of life and it is still the major cause of
epiglottitis.
The organism is resistant to penicillin, and ceftriaxone or cefotaxime is the
recommended treatment.
Non-typable H. influenzae (not HI type b) causes ear infections (otitis media)
and sinusitis in children.
Further reading:
Hib infection and Hib vaccination Centers for Disease Control and Prevention

40-Compared with intracellular fluid, extracellular fluid


True / False
Has a higher potassium concentration
Has a higher osmolarity
Has a higher pH

Correct

Correct

Incorrect answer selected

Has a higher protein concentration

Correct

Has a higher phosphate concentration

Compared with intracellular fluid, ECF has a

Incorrect answer selected

higher

sodium

chloride

calcium

pH (as intracellular metabolism results in lower


pH)

lower

osmolality

phosphate

protein concentration (obviously)

potassium concentration.

Reference:
Ganong's Review of Medical Physiology 23rd Edn 2010: p.3.
Silbernagl S, Despopoulos A. Color Atlas of Physiology 6th Edn 2009: p.9293.
Next question

41-A 17-year-old boy was brought to clinic as his parents were concerned
regarding possible delayed puberty.
He was otherwise well, played sports regularly and academic performance
was good. His height was 1.7 m and weight was 70 kg.
On examination he had small penis and testes, absent pubic hair, but no
other abnormalities.
Investigations revealed:
Serum testosterone

4 nmol/l

(9-35)

1 U/l

(1-7)

Plasma luteinising hormone (LH)

1 U/l

(1-10)

Plasma prolactin

300 mU/l

(<450)

Plasma TSH

2 mU/l

(0.5-5)

Plasma follicle stimulating hormone


(FSH)

Which one of the following is the most likely cause?


(Please select 1 option)
Constitutional delay

Incorrect answer selected

Hypopituitarism
Hypothyroidism
Kallman's syndrome

This is the correct answer

Klinefelter's syndrome

The low follicle-stimulating hormone (FSH) and luteinising hormone (LH),


together with the low testosterone, suggests a hypogonadotrophic
hypogonadism.
We know that there is no mental retardation, and we are told that physical
examination is normal and sense of smell would usually not be tested.
Consequently a diagnosis of Kallman's is suggested.
We are not told of a family history of growth delay, thus this is unlikely to be
constitutional delay.
The thyroid-stimulating hormone (TSH) is normal, making hypothyroidism
unlikely and this together with the normal prolactin make hypopituitarism
most unlikely.
Next question

42-A 12-year-old male with type 1 diabetes is seen at annual review.


His glycaemic control is suboptimal on diet alone and his most recent HbA1c
is 7.9% (3.8-6.4). You elect to change his current insulin regime.
Which of the following would be the most appropriate interval to re-check his
HbA1c following the alteration in therapy?
(Please select 1 option)

Two weeks
One month
Two - three months

Correct

Four - six months


Six - twelve months

The HbA1c is a reflection of the glycosylation of the haemoglobin moiety by


glucose.

There is a strong correlation between the glycosylation of this molecule and


average plasma glucose concentrations, hence its widespread use in clinical
practice as a tool to assess glycaemic control.
Furthermore, studies reveal its prognostic significance in both microvascular
and macrovascular risk.
The life span of the red cell is 120 days. HbA1c reflects average blood
glucose levels during the half-life of the red cell (about 60 days) and so the
recommended appropriate interval for re-measuring HbA1c following change
in therapy is two months.

43-The following are recognised treatments of acute severe asthma in a 4year-old girl:
True / False

Inhaled sodium cromoglycate therapy


IV salbutamol infusion
Nebulised steroids
Sedation

Incorrect answer selected

Correct

Incorrect answer selected

This is the correct answer

Unlimited humidified oxygen

Correct

The management of acute severe asthma has been codified in the regularlyupdated British Thoracic Society Guidelines (2009). You are strongly
recommended to familiarise yourself with these.
Calm and skilful management will reassure both parents and child.
Oxygen should be given, and nebulised beta-2 agonists commenced.
If nebulised treatment is unavailable, then 5-10 puffs of bronchodilator can
be given through a spacer device.
In severe asthma, intravenous (IV) aminophylline may be used. A loading
dose is given over 20 minutes followed by a continuous infusion.
A bolus may result in seizures, severe vomiting, and fatal cardiac
arrhythmias.
If the child is already on oral theophylline, then the loading dose should be
omitted. IV salbutamol can be used as an alternative.

Nebulised steroids have not been evaluated, but oral steroids are routinely
given for two to five days to expedite recovery.

44-Which of the following micro-organisms are generally sensitive to


benzylpenicillin?
True / False
Bordetella pertussis

Correct

Cryptococcus neoformans

Correct

Mycoplasma pneumoniae

Correct

Streptococcus pneumonaie
Streptococcus viridans

Correct

Correct

Penicillin binds to specific penicillin-binding proteins (PBPs) in the cell wall,


mainly of Gram-positive organisms.
Penicillin resistance is usually due to production of altered PBPs or betalactamases which leave the penicillin molecule.
Penicillin is mainly useful for group A Streptococcus, group B Streptococcus,
meningococcal and pneumococcal infections, though anthrax is also generally
sensitive.
Pneumococci with modified PBPs are an increasing problem.
Copyright 2002 Dr Colin Melville

45-The following mechanisms of microbial resistance are correctly ascribed:


True / False
Enterococcus faecalis by beta-lactamase production

Incorrect answer selected

Herpes simplex by mutations of viral thymidine kinase

Correct

Pseudomonas aeruginosa by mutation of specific binding proteins

Incorrect

answer selected
Staphylococcus aureus by slime production

Correct

Staphylococcus epidermidis by slime production

Correct

Pseudomonas produce inducible beta-lactamases and slime. Staphylococcus


aureus produces beta-lactamases.
Copyright 2002 Dr Colin Melville
Next question

46-Which of the following are characteristic of early tetanus?


True / False
Rigid abdomen

Incorrect answer selected

Rigid jaw muscles


Dysphagia

Incorrect answer selected

Correct

Hyperpyrexia

Correct

Carpopedal spasm

Correct

Infantile generalised tetanus develops within 0-3 days of birth.


The usual picture is of

poor feeding

hunger

crying

paralysis

decreased movement.

The child becomes stiff with muscular spasms and opisthotonus. A


contaminated umbilicus may be obvious.
The fever is usually secondary to muscular spasm (muscular energy).
Carpopedal spasms -/+ laryngospasm occur in manifest tetany due to
extreme vitamin D deficiency, hypomagnesaemia or hypocalcaemia. The
wrists are flexed with extended fingers and adducted thumbs with extended
and adducted feet.

Latent tetany refers to carpopedal spasm occurring on ischaemia of the


motor nerves caused by blowing up of a blood pressure cuff.
Copyright 2002 Dr Colin Melville

47-A 2-week-old girl presents with a history of poor feeding, worse over the
last six hours.
She was born at 38+3/40 weeks, weighing 2.95 kg, with no problems at
birth. It was the first pregnancy of a healthy Caucasian mother. There is no
FH/SH of note. She has had no drugs nor immunisations.
On examination temperature is 35.4C (tympanic), RR 60/min, HR 160/min
on monitor (all pulses impalpable). Cold mottled peripheries, capillary refill
time six seconds. O saturations 45% in air. Gallop rhythm, 4 cm liver, no
spleen. No bruising or rash.
2

What is the most likely diagnosis?


(Please select 1 option)
Congenital adrenal hyperplasia
Congenital heart disease

Correct

Inborn errors of metabolism


Sepsis

The presentation is with shock and a low temperature. Although all diagnoses
are possible, a cardiac lesion is most likely due to the gallop rhythm and 4
cm liver.
CAH at this age would usually be due to salt loss. The liver would not be
enlarged, and there would be no murmur except perhaps a flow murmur.
Inborn errors tend to be associated with coma, acidosis and/or
hypoglycaemia. Liver enlargement can also occur. Fortunately, these
conditions are quite rare.
Sepsis should always be considered. Hypothermia does not exclude this, but
a large liver makes it less likely.
Shaken babies can present with hypothermia, coma and poor perfusion.
There may be external skin bruising, fractures, and retinal haemorrhages
too. There is not usually a murmur nor hepatomegaly.

48-In nephrotic syndrome, complete resolution is unlikely in the presence


of:
True / False
Highly selective proteinuria
Hypertension

Correct

Correct

Macroscopic haematuria

Correct

Proliferate glomerular changes on electron microscopy


24 hour urine protein >10 g/l at presentation

Correct

Incorrect answer selected

The nephrotic syndrome is characterised by

proteinuria

hypoproteinaemia

oedema

hyperlipidaemia.

Ninety per cent have idiopathic nephrotic syndrome (of these, 85% have
minimal change, 5% mesangial proliferative, and 10% focal sclerosis).
In the remaining 10%, the nephrotic syndrome is mediated by some form of
glomerulonephritis, with the commonest causes being membranous and
membranoproliferative.
Minimal change nephrotic syndrome has the best prognosis, and this is
associated with 24 hour urine loss of more than 2 g, composed primarily of
albumin.
Hypertension is uncommon, though there may be slightly reduced creatinine
clearance and microscopic haematuria.
The presence of macroscopic haematuria suggests an associated nephritic
element, and therefore associated glomerulonephritis with a worse prognosis.
Proliferative changes suggest a similar abnormality. Hypertension is also
characteristic of nephritic syndrome. These are therefore associated with a
worse prognosis.
Copyright 2002 Dr Colin Melville

49-Regarding the diagnosis of urinary tract infection in a 3-month-old child,


which of the following statements is/are true?
True / False

A bag urine will confirm it

Correct

DMSA scan should be performed in confirmed cases


IVP is the only investigation required in most cases
MCUG should be performed in suspected cases

Correct
Correct

Incorrect answer selected

Ultrasound scan of kidneys, ureters and bladder should be performed in all


suspected cases

Correct

The diagnosis of urinary tract infection in infants is difficult.


A bag specimen is only useful when negative to exclude infection. At least
two bag urines growing a pure growth of more than 105 of the same
organism with the same sensitivities, accompanied by pyuria can be taken as
presumptive evidence of an infection.
However, the gold standard remains the suprapubic aspirate or catheter
specimen of urine.
In the former, any number of Gram negative bacteria indicates infection,
though a small number of Gram positive cocci may represent skin
contaminants.
The absence of pyuria does not rule out infection.
In infancy, 33% of blood cultures are positive in those with proven urinary
tract infection, and meningitis can also occur simultaneously.
An ultrasound scan should be performed in all confirmed cases soon after the
diagnosis. This is helpful in ruling out a pyonephrosis or renal abscess.
After resolution, a micturating cystourethrogram (MCUG) is done to exclude
reflux, and a dimercaptosuccinic acid (DMSA) scan can help in diagnosing
pyelonephritis or scarring.
An intravenous pyelogram (IVP) is only done in selected cases, though
historically this was the popular investigation.

Further reading:
Urinary tract infection in children NICE guidelines 2007
Copyright 2002 Dr Colin Melville
Next question

The following are features of late iron poisoning:


True / False
Abdominal pain
Convulsions

Incorrect answer selected

Correct

Gastrointestinal bleeding
Shock

Incorrect answer selected

Correct

Vomiting

Incorrect answer selected

There are five phases of serious iron poisoning:


1. Local irritation of the gastric mucosa (half to 2 hours,
subsiding by 12 hours), nausea, vomiting, diarrhoea,
abdominal pain, haematemesis and bloody diarrhoea may
occur with severe hypotension.
2. (Next 2-6 hours) Apparent recovery. During this time, iron
accumulates in mitochondria and various organs.
3. (12 hours post-ingestion) Cellular damage produces
hypoglycaemia and metabolic acidosis. Anaerobic
metabolism and interference with Kreb cycle results in
lactate acidosis.
4. (2-4 days after ingestion) Severe hepatic necrosis, and
abnormal liver function tests with lowered prothrombin.
5. (2-4 weeks post-ingestion) Scarring and stenosis of the
pyloric area, occasionally requiring surgical intervention.

The first three are, therefore, early signs, while vomiting may occur because
of pyloric stenosis, and convulsions from hepatic coma. GI bleeding, shock
and abdominal pain can also occur due to late complications.

Copyright 2002 Dr Colin Melville

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