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1-A 14-year-old boy presents with pink urine following a boxing match.

Full term
normal delivery with no neonatal complications. Immunisations up to date. There
is no FH/SH of note.
On examination he is apyrexial and well. Respiratory rate 12/min and pulse
65/min. Tender in the left loin on bimanual palpation.
What is the most likely diagnosis?
(Please select 1 option)

Adult polycystic kidney disease


Benign familial haematuria
Post-streptococcal glomerulonephritis
Trauma

Correct

Urinary tract infection


The history of haematuria and finding of loin pain suggests renal trauma. The extent of
damage can be assessed by USS of KUB. Minor trauma will usually settle spontaneously.

2-A 3-year-old boy goes to a children's party and eats some peanuts. Almost
immediately he spits them out, and runs to his mother saying his mouth hurts.
She notes a rapidly developing raised itchy rash over his face, and that his eyes,
lips and tongue are swelling. He begins to have marked difficulty in breathing with
audible stridor. An ambulance is called.
On the arrival of the paramedics he has collapsed and appears unconscious. He
has shallow breathing at 10/min, HR 160/min (thready pulse), and is responding
only to pain.
What is the most likely diagnosis?
(Please select 1 option)

Acute severe asthma


Anaphylaxis

Correct

Croup
Foreign body aspiration
Trauma
The history is of anaphylactic shock in response to peanuts. After attention to airway and
breathing he requires IM adrenaline, then IV steroids and antihistamines and nebulised
salbutamol. Fortunately anaphylactic shock is rare.

3-A 4-year-old girl presents with extensive bruising over the trunk, legs and
palate. She had an URTI 4 weeks ago, but has otherwise been well. 39+5/40
3.8kg delivery, with no neonatal problems. No FH of note.
On examination she is well. Temperature is 36.7C (tympanic), HR 90/min, RR
20/min. Extensive fresh bruising of irregular shape over trunk, thighs and shins.
Old bruises over shin.
What is the most likely diagnosis?
(Please select 1 option)

Acute lymphoblastic leukaemia


Child physical abuse
Henoch Schoelein purpura
Idiopathic Thrombocytopaenic Purpura

Correct

Meningococcal septicaemia
This girl had a presumed viral illness a month ago and now presents with sudden onset of
extensive bruising. The likely diagnosis is Idiopathic thrombocytopaenic purpura. This can
be confirmed by an FBC and film, which shows isolated thrombocytopaenia, often
<10X1012/l. Most can be managed conservatively, with the platelet count recovering
spontaneously over 1-4 weeks. IVIG or steroids may accelerate recovery. If steroid therapy
is contemplated it should be preceded by a bone marrow biopsy to exclude an
thrombocytopaenic presentation of leukaemia.

4-A 9 month old boy presents with a 24 history of fever and poor feeding. He
has become less responsive and has developed a purple rash over his arms and
trunk. 40+2/40, 3.03kg, no neonatal problems. No drugs. Fully immunised. No
FH or social history of note.
On examination temperature is 38.2C, RR 50/min (mild recession, HR 150/min.
Cool peripheries, with capillary refill time of 4 seconds. Non-blanching purple
spots 3-27mm over the arms and trunk.
What is the most likely diagnosis?
(Please select 1 option)

Child physical abuse


Henoch Schoelein purpura
Idiopathic Thrombocytopaenic Purpura
Meningococcal septicaemia

Correct

Pneumococcal septicaemia
The history is brief, with a sick, shocked child with purpura. The diagnosis is purpura
fulminans, probably due to septicaemia. The 3 commonest organisms which cause this are
meningococcus (esp. B, A and C), pneumococcus and Haemophilus influenzae (esp. B).

The incidence of Meningitis C and Hib have declined dramatically since the introduction of
routine immunisations.

5-A 9-year-old girl presents with a history of clear nasal discharge for several
weeks, accompanied by sneezing and red eyes. This has happened for the last 2
spring times. Full term normal delivery, no neonatal problems. Immunisations up
to date. No family or social history of note.
On examination she is apyrexial and well. She has clear rhinorrhoea, is constantly
rubbing her nose and eyes, and has bilateral non-purulent conjunctivitis. She
breathes through her mouth.
What is the most likely diagnosis?
(Please select 1 option)

Allergic rhinitis

Correct

Nasal foreign body


Sinusitis
Vasomotor rhinitis
Viral URI
The history of seasonal sneezing, nasal discharge and eye irritation suggests an allergic
rhinitis. The timing depends on the allergens, but typically is Spring time for wind-borne
tree and weed pollens. Treatment is with antihistamines or topical steroids.

6-A 6-year-old girl presents with cervical lymphadenopathy of 3 months


standing, which has increased progressively in size. She has been off colour with
a chronic cough and hoarse voice. Full term normal delivery, no neonatal
problems. Immunisations up to date. No family or social history of note.
On examination the temperature is 36.8C, respiratory rate 20/min and pulse
85/min. Looks unwell and thin. 3rd centile for weight, 25% for height. Massive
rubbery enlargement of right cervical glands, which are non-tender. No overlying
erythema. Otherwise well.
What is the most likely diagnosis?
(Please select 1 option)

HIV
Infectious mononucleosis
Kawasaki disease

Lymphoma

Correct

Mycobacterial adenitis
The progressive enlargement of nodes over several months without associated
inflammation suggests malignant infiltration. The abnormal voice suggests involvement of
the recurrent laryngeal nerve. Assessment will require chest X-ray and MRI or CT of neck
and thorax, and lymphnode biopsy. Prognosis will depend on staging and histology
(including molecular tests).

7-A 15 month old boy presents with high fever of 6 days duration, rash, tender
neck glands, red eyes and cracked lips. Full term normal delivery with no
neonatal complications. Immunisations up to date. There was no FH/SH of note.
On examination:
temperature

38.9C

Respiratory rate

30/min

Heart rate

120/min

The rash is maculopapular erythematous and blanching. The hands and feet are
swollen. He has tender cervical gland enlargement, non-purulent conjunctivitis
and prominent tongue papillae. He is miserable and clingy
What is the most likely diagnosis?
(Please select 1 option)

Kawasaki disease

Correct

Measles
Rubella
Scarlet fever
Toxic shock syndrome
The history is classical of Kawasaki disease (muco-cutaneous lymphnode syndrome). The
features described fulfil the international criteria, the diagnosis being purely clinical. Often
there is elevation of CRP, ESR and platelet count. IVIG 2g/kg should be given within the
first 10d of illness to reduce the risk of coronary artery aneurysm.

8-A 17-year-old male presents to A+E after an overdose of alcohol and


paracetamol. He complained of abdominal discomfort and an intravenous infusion
of N-Acetylcysteine was commenced. 15 minutes later he developed
breathlessness, reported feeling flushed and developed a tachycardia.
What is the most likely explanation for this reaction?

(Please select 1 option)

A disulfiram-like (antabuse) reaction has occurred


The patient has had a panic attack
The patient has developed pulmonary oedema

Incorrect answer selected

The patient has received an overdose of N-Acetylcysteine


The patient has received N-Acetylcysteine previously

This is the correct answer

This patient is having an acute hypersensitivity reaction the most common, dose
independent adverse drug reaction. It is caused by previous exposure and being
sensitised to the drug. The initial exposure induces the production of antibodies of
Ig E class, subsequent exposure induces an immunological reaction - anaphylaxis.
Some drugs can produce an similar pseudoallergic reaction on first exposure.

9-A 10-year-old boy presents with a history of diarrhoea over the past 3 months.
This is loose, occasionally admixed with blood, and occurs up to 4 times per day.
He has felt unwell and lacking in energy, and has occasional griping abdominal
pain. Previous history is unremarkable. He is fully immunised and on no
medications. There is no FH/SH of note.
On examination he is pale and has early clubbing. He is on the 25% for height
and the 3% for weight. Temperature is 37.2C, Respiratory rate 15/min and pulse
80/min. The abdomen is diffusely tender and anal examination reveals an anal
fissure and small tag.
What is the most likely diagnosis?
(Please select 1 option)

Coeliac disease
Crohns disease

Correct

Perianal abscess
Shigella gastroenteritis
Ulcerative colitis
The chronic bloody diarrhoea, systemic upset, pallor, clubbing and perianal findings make
Crohn's disease the likeliest diagnosis. This can be confirmed histologically by upper and
lower GI endoscopy and biopsy (non-caseating granulomata). Barium series help to define
the extent of disease, which can affect from the mouth to the anus.

10-A 14-year-old girl was found unconscious at the roadside by a passer-by,


who called an ambulance.

On examination she was unkempt, had slurred speech and an ataxic gait. Her
core temperature was 34.5C, but there were no other specific abnormalities to
find. Her BM stix result was 2.9 mmol/l.
What is the most likely diagnosis?
(Please select 1 option)

Alcohol ingestion

Correct

Complex partial seizure


Encephalitis
Insulin overdose
Migraine

The picture suggests acute alcohol ingestion. This is often accompanied by


hypothermia and hypoglycaemia.
It is important to exclude other ingestions and to look for coexisting social
problems.

11-A 2-year-old girl presents with a raised itchy rash. She had been eating a
chocolate bar when she spat it out saying it hurt her mouth. Within minutes the
rash had appeared over her face and trunk. She was 39+1/40 weighing 3.25kg
and there were no neonatal problems apart from cradle cap. She has been fully
immunised to date. There is a family history of asthma.
On examination she has a florid raised rash over her face. Some of the lesions are
coalscing. She is scratching, but breathing and tongue size are normal. Her
temperature is 36.9C (tympanic), Respiratory rate 30/min, and heart rate of
100/min.
What is the most likely diagnosis?
(Please select 1 option)

Atopic dermatitis
Contact dermatitis
Impetigo
Scabies
Urticaria

Correct

The history suggests an urticarial reaction to one of the components of the chocolate bar.
Manufactured foods make it difficult to identify the offending substance. Treatment is with
antihistamines prn and avoidance of known precipitants.

12-A 16-year-old male presents with acute severe asthma. On examination his
peripheral pulse volume fell during inspiration.
Which one of the following is the most likely explanation for this clinical sign?
(Please select 1 option)

The cardiac effect of high dose beta agonist bronchodilator drugs


A falling heart rate on inspiration
Myocardial depression due to hypoxia
Peripheral vasodilatation
Reduced left atrial filling pressure on inspiration

Correct

This patient is demonstrating pulsus paradoxus. The right heart responds directly
to changes in intrathoracic pressure, while the filling of the left heart depends on
the pulmonary vascular volume. At high respiratory rates, with severe air flow
limitation (eg acute asthma) there is an increased and sudden negative
intrathoracic pressure on inspiration and this will enhance the normal fall in blood
pressure.

13-A 16-year-old girl presents with a 2 day history of deteriorating


breathlessness and dyspnoea. Blood gas analysis shows a pH of 7.25, a PCO2 of
7.0kPa, a PO2 of 8.5kPa, and a base excess of -4. Which of the following
interpretations is correct?
(Please select 1 option)

Results are consistent with bronchopulmonary dysplasia.


Blood gases suggest type 1 respiratory failure.
Immediate intubation is required.
Results are consistent with late severe asthma.

Correct

Bicarbonate may be necessary to correct the acidosis.

In interpreting blood gas results, the following sequence may be useful:

Inspect the pH: Is it low, normal or high?

Inspect the CO2: Is it low, normal or high?

Inspect the PO2: Is it low, normal or high?

If the pH is low then an acidosis is present, and inspecting the CO2 will enable you
to determine whether this is due to respiratory or metabolic causes. Inspecting
the PO2 will tell you whether the patient is hypoxic or not. In this case, the pH is
reduced, and the CO2 is high, with a base deficit of only -4, insufficient to explain

the acidosis from metabolic causes. This is, therefore, a respiratory acidosis, and
the PO2 is also low suggesting type 2 respiratory failure. Possible causes would
include severe pneumonia, end stage asthma or neurogenic causes such as
guillain-Barre. In asthma, the initial stages show a low CO2, with this climbing
only to accompany failing respiration. The results would therefore be consistent
with late severe asthma. In bronchopulmonary dysplasia, there is usually longterm CO2 retention with compensatory increase in bicarbonate leading to a
positive base excess and normal pH. Bicarbonate is usually only considered if the
base deficit exceeds about -8.

14-A 16-year-old girl is diagnosed with Turner's syndrome. Which of the


following autoimmune conditions is most commonly associated with Turner's?
(Please select 1 option)

Addison's disease
Autoimmune hepatitis
Hashimoto's thyroiditis

Correct

Sjogren's syndrome
Vitamin B12 deficiency

Hypothyroidism is quite common occurring in up to 24% of patients with Turner's


syndrome. It is typically autoimmune in origin - Hashimoto's thyroiditis though
the exact explanation for its high prevalence is not known.

15-A 17-year-old boy whose brother had hypertrophic cardiomyopathy was


referred for a cardiological assessment. His echocardiogram confirmed the
condition.
Which one of the following echocardiographic features is the most important risk
factor for sudden cardiac death?
(Please select 1 option)

A gradient of 10 mmHg across the left ventricular outflow tract


Significant thickening of the interventricular septum

Correct

An enlarged left atrium


Systolic anterior motion of the mitral valve
The presence of mitral regurgitation

In hypertrophic obstructive cardiomyopathy the cause of death is usually


ventricular tachycardia or ventricular fibrillation and therefore the thicker the

muscle the more abnormal the cardiac architecture and the higher the risk of
arrhythmia and sudden death.

16-A 7 month old boy presents with a history of cough, fever and difficulty in
feeding for 3 days. He has become progressively more breathless. On admission
he has a fever to 38.9C and looks unwell. His respiratory rate is 65/min with
moderate recession. FBC showed a White cell count of 25.7 x109/L (4-11 x109)
[82% neutrophils]. The C-reactive protein was 145 mg/L (<10).
What is the most likely diagnosis?
(Please select 1 option)

Aspiration pneumonia
Asthma
Bronchiolitis
Cystic fibrosis
Pneumococcal pneumonia

Correct

The picture is suggestive of a bacterial infection. The rapid respiratory rate


suggest a lower respiratory infection, with pneumonia the number one suspect.
Most bacterial cases are caused by pneumococcus and respond to penicillin.

17-A 12-year-old afro-caribbean girl presents with fever, malaise, and joint
pains of 3 months duration. She was previously well. Full term normal delivery, no
neonatal problems. Immunisations up to date. No family or social history of note.
On examination the temperature is 37.6C, respiratory rate 14/min, pulse 80/min
and Blood Pressure is 100/70 mmHg. She appears subdued and well perfused.
She has a scanty rash over her cheeks. She has generalised joint tenderness on
extremes of movement, but there is no obvious effusion not warmth in them. She
has 2+ haematuria and 2+ proteinuria.
What is the most likely diagnosis?
(Please select 1 option)

Chronic multifocal osteomyelitis


Reactive arthritis
Septic arthritis
Sickle cell crisis
Systemic lupus erythematosis

Correct

The prolonged history of fever, malaise, joint pains, malar rash and renal involvement
point to a multisystem disease. In this ethnic group SLE is the most likely diagnosis. This
can involve any system and is a great mimic. Treatment is with NSAIDs for joints plus
immunosuppressives for the renal involvement. Therapy may be difficult and prolonged.

18-A 5-year-old boy presents with recurrent ear pain and fever. Every time he
gets a cold he develops ear pain and receives antibiotics from the GP. He has
about 6 episodes a year. He was born at term weighing 2.9kg and there were no
neonatal problems. He is fully immunised. Parents are both smokers.
On examination he has a temperature of 38.0C, respiratory rate of 15/min and
pulse of 90/min. He has shotty slightly tender cervical nodes. His left eardrum is
red and bulging. Throat examination is normal.
>p>What is the most likely diagnosis?
(Please select 1 option)

Group A streptococcal pharyngitis


Mastoiditis
Otitis media with effusion
Otitis media, acute

Incorrect answer selected

Otitis media, recurrent

This is the correct answer

The history suggests recurrent episodes of acute otitis media with each URTI. If these are
progressively causing damage (eg eardrum scarring) prophylactic antibiotics and
investigations for minor immune deficiency may be considered. Most improve with age.

19-A 12 day old girl presents with poor feeding, vomiting and lethargy. Born at
37+6/40, 2.98kg, no neonatal problems. Uncomplicated pregnancy and delivery.
No drugs or medications. No immunisations. No FH/SH of note.
On examination she is thin and wasted. She has occasional twitching of the
eyelids and mouth. She responds slightly to voice. Weight <3%, OFC 25%,
temperature is 36.5C (tympanic), RR 40/min, HR 150/min. Chest clear, no
murmur. No organomegaly. No rash or dysmorphic features.
What is the most likely diagnosis?
(Please select 1 option)

Congenital adrenal hyperplasia


Congenital heart disease
Inborn errors of metabolism

Correct

Sepsis

This child is dehydrated, vomiting and lethargic. Examination suggests possible


subtle fits. Although infection and a cardiac abnormality are possible, a metabolic
condition should be strongly suspected. Evaluation should include electrolytes,
NH4, blood gases, serum and urine aminoacids, and urine organic acids.

20-A 17 month old boy has been completely well and playing with his toys.
Mother hears him coughing and choking and rushes next door. She finds him
gagging and retching, drooling and red in the face. He was born at 37+3/40
weighing 3.7kg and there were no neonatal problems.
On examination his temperature is 36.9C, RR 30/min, HR 130/min. He looks
slightly cyanosed with an O2 saturation of 85% in air. He has marked stridor and
moderate recession.
What is the most likely diagnosis?
(Please select 1 option)

Retropharyngeal abscess
Foreign body aspiration

Correct

Anaphylaxis
Croup
Peritonsillar abscess

The history suggests aspiration of a foreign body. Small objects, such as toys, are
the commonest offenders. They can give rise to acute obstruction, or there may
be a latent period when symptoms settle before inflammation around the object
gives rise to symptoms. Assessment requires a chest X-ray, which will locate
radioopaque objects, and bronchoscopy to remove the object.

21-A 4-year-old girl develops a fever 14 day after returning from a family visit
to India. At the height of fever she feels shivery and cold. She has previously
been well. Full term normal delivery with no neonatal complications.
Immunisations up to date. There is no FH/SH of note.
On examination she has a fever to 39.8C, Respiratory rate 20/min and pulse
100/min. Chest and ENT examinations are normal. She is shivering violently. She
has 3 cm spleen, which is smooth and non-tender.
What is the most likely diagnosis?
(Please select 1 option)

HIV infection
Infectious mononucleosis
Kala-azar
Malaria

Correct

Schistosomiasis

The history of fever, rigors and splenomegaly in a child returning from an endemic
area suggests malaria. Thick and thin blood smears should be taken. Malarial
resistance is an increasing problem, and the latest information should be obtained
about the area of disease acquisition to ensure proper treatment.

22-A mother is concerned because her one-year-old girl was in contact with measles 2 days
previously. Which one of the following statements is correct.
(Please select 1 option)

Nothing can be done to affect the course of the disease.


Gamma globulin can be administered up to 14 days after exposure.
If she were to become infected then complications would be extremely rare.
Immunisation with the live attenuated virus will give long term immunity if given
immediately

Correct

She should anticipate the rash occurring in the next 5 days.


If the person is within 72 hours of exposure measles vaccine is the treatment of choice as
it will confer life long immunity. In persons in whom the vaccine is contraindicated immune
globulin can be given. Immune globulin is effective within 6 days of exposure. One third of
those afflicted with measles can expect a complication. These include pneumonia, otitis
media, diarrhoea. A serious complication is the development of sub acute sclerosing, panencephalitis. The initial symptoms of measles are corysal symptoms. Several days after the
appearance of corysal symptoms a rash appears.

23-Which one of the following is a characteristic feature of hereditary


spherocytosis?
(Please select 1 option)

It is transmitted as an autosomal recessive trait


It does not present during the neonatal period
In a 5-year-old child the spleen is likely to be enlarged
The Coombes test is usually positive

Correct

The majority of those affected will die before reaching adult life

The disease is usually transmitted in an autosomal dominant fashion. Severe


forms can present in the neonatal period . Splenomegaly is common. Haemolysis
occurs and the blood film can look similar to autoimmune haemolytic anaemia.
The Coombes test is negative in hereditary spherocytosis which helps distinguish
the two conditions. Splenectomy after the age of 5 stops further haemolysis and
the patient can lead a normal life.

24-A 1-year-old male child is suspected to have acute lymphoblastic leukaemia.


Which one of the following correctly applies:
(Please select 1 option)

there is likely to be a family history of ALL


he is likely to have an abnormality on chromosome 21
his age suggests he is in the good prognosis group
the condition does not affect the meninges
male sex is associated with adverse prognosis

Correct

ALL is the most common representing 23% of childhood cancers, with an annual
rate of 30-40 per million. There is no genetic predisposition. Age of less than 2
and greater than 9 at presentation suggests adverse prognosis.The chromosomal
abnormality in ALL involves a translocation in the 11q23 region.It does affect the
meninges which is why prophylactic cranio-spinal irradiation and intrathecal
methotrexate is given. It carries a good prognosis with a cure rate of 80%.
However male sex carries a worse prognosis.

25-Which one of the following is a cause of delayed bone age:


(Please select 1 option)

Central precocious puberty


Congenital hypothyroidism in a 6-year-old boy treated with thyroxine since 7
days old
Newly diagnosed adrenal hyperplasia in a 5-year-old boy
Newly diagnosed growth hormone deficiency in a 6-year-old girl

Correct

Obesity

Bone age would be normal in an adequately treated euthyroid child. Bone age
would be delayed in growth hormone deficiency. In exogenous obesity due to
overnutrition and underexercise bone age would be advanced. If there is a
concomitant endocrinopathy such as hypothyroidism then bone age would be

delayed. Bone age is delayed by 2 years in childhood in Turner's syndrome. CAH


and central precocious puberty cause advanced bone age.

26-A 17-year-old girl underwent emergency splenectomy after a domestic


accident. Which one of the following organisms is most likely to cause lifethreatening infection in the future?
(Please select 1 option)

Actinomycosis
Haemophilus influenzae

Incorrect answer selected

Pseudomonas aeruginosa
Staphylococcus aureus
Streptococcus pneumoniae

This is the correct answer

Following splenectomy a person is at risk of Streptococcus pneumoniae ,


Haemophilus influenzae, Nesseria meningitidis, Escherichia coli and Pseudomonas
aeruginosa.
By far the most common is Streptococcus pneumoniae- which can cause life
threatening infection.

27-A 18 year-old man is referred with a 6 month history of daily headache


which is mostly frontal in location and occasionally associated with nausea. He
has been taking Paracetamol 3g/day, Aspirin 300 mg tds and Codeine 40mg tds,
which has only a temporary effect. He has a 2 year history of depression, treated
with Paroxetine. No abnormalities were found on examination. What is the most
likely diagnosis?
(Please select 1 option)

Analgesic misuse headache

Correct

Cerebral tumor
Cluster headache
Headache due to depression
Migraine

The two commonest causes of chronic daily headache are tension type headache
and analgesic misuse headache. The latter is the most likely diagnosis for this
patient's symptoms. It usually occurs as a result of chronic use of analgesics such
as codeine phosphate and paracetamol. Typically the headache is relieved with

the analgesics just to return in the next hours. The treatment of choice is the
slow reduction and withdrawal of analgesics.

28-Which of the following organelles contains enzymes responsible for the


digestion of constituents of cells and tissues?
(Please select 1 option)

endoplasmic reticulum
Gogli apparatus
lysosomes

Correct

microtubules
mitochondria

The lysosomes contain the enzymes and molecules such as oxidases, free radical
etc responsible for the breakdown of intracellular components. Micortubules are
involved in mitotic processes and intracellular transportation. The mitochondria
produce energy for cellular functions.

29-A 17-year-old male from India presents with fever of 4 months duration and
splenomegaly. What is the most likely diagnosis?
(Please select 1 option)

Coccidiomycosis
Giardiasis
Tropical sprue
Typhoid
Visceral leishmaniasis

Correct

Visceral leishmaniasis (Kala-azar) is an endemic disease in several regions of India and


sub-Saharan Africa. It is caused by the parasite Leishmania donovani donovani and spread
by Phlebotomus sand-flies. Leishmaniasis is common in immune-suppressed patients,
particularly those infected with HIV. There has recently been a substantial increase of
cases in the Mediterranean region. It has been estimated that 15% of HIV positive drug
users in Spain are infected with Leishmania donovani infantum. Giardiasis and tropical
sprue present with gastrointestinal symptoms and malabsorption. Typhoid is an acute
illness. Coccidiomycosis is largely confined to the Americas. Most patients present with
pulmonary symptoms although disseminated disease can occur particularly in the immunesuppressed.

30-Which of the following concerning IgG is correct?


(Please select 1 option)

It has a molecular weight of 50,000 kd.


It is monovalent.
It comprises the majority of circulating antibody in serum.

Correct

It differs from other isotypes in not being able to cross the placental barrier.
It is the major antibody produced during the primary response.
a) Each light chain has a MW of 25,000 and each H chain a MW of 50,000. Therefore, since
the whole molecule consists of 2 L and 2 H chains, the MW is 150,000 kd. b) It exists as a
monomer with 2 Fab portions, each of which can interact with an antigenic determinant.
Therefore it is divalent. c) Normal range 8-19 g/l. Next is IgA, 1-5 g/l, followed by IgM
0.5- 2 g/l. d) It is in fact the only antibody capable of crossing the placental barrier, which
it does through gaining attachment via its Fc portion. e) It is the major antibody produced
in the secondary immune response. IgM is the major antibody produced during the
primary response. (c) Dr Alan Cann

31-A 17-year-old girl is short in stature for her age. She has not shown any
changes of puberty. She has a webbed neck. Her vital signs include Temperature
36.6C Respiratory rate 18/min Pulse 75 bpm and BP 165/85 mmHg. On physical
examination, she has a continuous murmur heard over both the front of the chest
as well as her back. Her lower extremities are cool with poor capillary filling. A
chest radiograph reveals a prominent left heart border, no oedema or effusions,
and rib notching. Which of the following pathologic lesions best explains these
findings?
(Please select 1 option)

Constriction of the aorta past the ductus arteriosus

Correct

Lack of development of the spiral septum and partial absence of conus


musculature
Shortening and thickening of chordae tendineae of the mitral valve
Single large atrioventricular valve
Supravalvular narrowing in the aortic root

She has coarctation of the aorta, and the constriction is postductal, allowing
prolonged survival. Her physical characteristics also suggest Turner syndrome
(monosomy X).

32-Which of the following is a polygenic disorder?


(Please select 1 option)

Ankylosing spondylitis

Correct

Erythropoietic porphyria
Fragile X syndrome
Huntington's disease
Pendreds syndrome

Unlike the other conditions, no one specific genetic defect has been identified to
account for ankylosing spondylitis. Hungtindon's chorea is an autosomal dominant
condition. Fragile X syndrome is due to a trinucleatide repeat at the FMR 1 gene
on the X chromosome. Erythropoietic porphyria is an autosomal recessive
condition as is Pendred's syndrome.

33-A 17-year-old male who appears tall and thin for his age, presents with a
high arch palate, chest wall deformities and livedo reticularis. Which of the
following is also associated with this syndrome?
(Please select 1 option)

Autosomal Dominance
Methionine accumulation

This is the correct answer

Osteopetrosis
Positive Guthrie test
Upward dislocation of the lens

Incorrect answer selected

This syndrome is most likely to be homocystinuria. Marfan's syndrome is


associated with upward dislocation of the lens, but not with livedo reticularis,
which is seen in Homocystinuria due to the venous thrombosis in the small
vessels of the skin. Homocystinuria is associated with downward dislocation of the
lens, and is an Autosomal Recessive disorder. Osteoporosis, and not osteopetrosis
is seen in homocystinuria. A positive Guthrie testis associated with
Phenylketonuria. Reduced activity of cystathionine -synthase results in
accumulation of homocysteine and methionine, interfering with collagen crosslinking, which is the cardinal feature of Homocystinuria.

34-Which of the following is associated with abnormalities of phosphorylation of


gene product by cyclin-dependent kinases (CDK); binding of gene product to
transcription factors such as E2F?
(Please select 1 option)

Retinoblastoma

This is the correct answer

Achondroplasia

Incorrect answer selected

Neurofibromatosis type 1
Huntington disease
Marfan syndrome

The retinoblastoma gene product is phosphorylated by a CDK and then binds to


transcription factors.
Marfan patients have mutations of the chromosome 15 gene encoding fibrillin, a
connective tissue protein.

35-A 14-year-old boy falls awkwardly while swinging from a rope in an


adventure playground. He lands head first, causing violent flexion of his neck. An
ambulance is called. He has previously been fit and 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. He has been intubated and is being
bag ventilated in 100% Oxygen. Heart rate is 60/min and pulse is thready,
though the peripheries are warm and pink. Blood pressure is 75 mmHg systolic by
dynamap. He is paralysed from the neck down, and has a GCS of 8.
What is the most mechanism of shock?
(Please select 1 option)

Anaemic
Cardiogenic
Distributive

Correct

Hypovolaemic
Septic
The history suggests spinal cord transection causing neurogenic shock. Severe head injury
or cord transection can prevent autonomic control of blood vessels, leading to fluid loss
into the tissues (distributive shock). Unusually for shock the pulse is slow and the
peripheries warm. Fluid replacement and treatment of the underlying cause (if possible) is
indicated.

36-A 7-year-old boy is stung by a wasp. He develops marked redness and


swelling at the site of the sting, then inspiratory stridor, then he collapses.
He was a full term normal delivery with no neonatal complications. His
immunisations are up to date. There is no FH/SH of note.
On examination he is unconscious but self-ventilating at 16/min. Oxygen
saturations are 94% in 100% facemask oxygen. He has mild stridor but minimal
recession, and looks generally swollen over the face and trunk.
What is the most important element of management?
(Please select 1 option)

Adrenaline IM

This is the correct answer

Chlorpheniramine IV
Hydrocortisone IV
Intubation

Incorrect answer selected

Salbutamol nebulised

The history is of rapidly progressive throat narrowing, followed by collapse,


precipitated by a wasp sting.
The diagnosis is anaphylaxis, due to an extreme immunoglobulin (Ig)E mediated
reaction, causing intravascular fluid depletion and circulatory collapse.
Intramuscular (IM) adrenaline, IV hydrocortisone, nebulised salbutamol and IV
chlorpheniramine may all be needed.
Specific desensitisation is possible, through an extended course of injections, but
avoidance is best. He should carry a Medicalert bracelet and the school and
parents instructed in the use of adrenaline autoinjectors.

37-A 2-year-old boy presents with neck glands and general unwellness for the
past month. Mother has also noted that 'his eyes look funny'. Full term normal
delivery, no neonatal problems. Immunisations up to date. No family or social
history of note.
On examination the temperature is 36.7C, with RR 25/min and HR 100/min. He
looks unwell and has marked rubbery cervical lyphadenopathy. The right eye has
a small pupil, which fails to dilate in the dark.
What is the most likely diagnosis?
(Please select 1 option)

Cerebral abscess
Cerebral TB
Cerebral toxoplasmosis
Lymphoma
Neuroblastoma

Correct

The history of massive glandular enlargement plus Horner's syndrome suggest a


neuroblastoma. About 30% of neuroblastomas arise in the thoracic or cervical ganglia.
Other rare presentations include "dancing eyes dancing feet" (opsoclonus-myoclonus) and
"racoon eyes" (proptosis and periorbital ecchymosis).

38-If a female carrier of a completely expressed X-linked condition marries an


asymptomatic male, which one of the following statements is statistically true of
their offspring:
(Please select 1 option)

Half of all children will be symptomatic


Half of the females will be carriers

Correct

Half of the males will be asymptomatic carriers


Half of the females will be asymptomatic
None of the males will be symptomatic

The 50% of males who carry a copy of the abnormal gene, passed down from
their mother, will express the disease. The other 50% will be asymptomatic. All of
the females will be asymptomatic, with 50% carrying the abnormal gene.

39-An 18 month old boy investigated for failure to thrive and delayed development is found
to have primary hypothyroidism. Which of the following statements applies to his condition.
(Please select 1 option)

He is unlikely to achieve normal intelligence despite immediate commencement


of appropriate treatment

Correct

If his bone age was 3 months the onset of his hypothyroid state is most likely to
have occurred at 3 months of age

Growth hormone deficiency is likely also to be associated


Hashimotos disease is the most likely cause.
He is more prone to develop parathyroid insufficiency
The diagnosis of congenital hypothyroidism has been delayed and hence thyroxine therapy
will not restore mental function fully. The hypothyroidism is likely to have been present at
birth. Growth hormone deficiency may be seen in association with secondary
hypothyroidism if pituitary insufficiency exists, but not in primary hypothyroidism. The
most likely aetiology is absent or ectopic thyroid. Hypoparathyroidism is unrelated.

40-Which of the following is commonly associated with congenital hypothyroidism in the


neonatal period:
(Please select 1 option)

hypothermia

This is the correct answer

Diarrhoea
Prolonged conjugated hyperbilirubinaemia

Incorrect answer selected

A small anterior fontanelle


Inguinal hernia
Hypothermia results from reduced metabolic rate. Constipation rather than diarrhoea is a
common clinical feature. Prolonged conjugated hyperbilirubinaemia is a relatively
uncommon sequelae. The anterior fontanelle would be tense. Umbilical rather than inguinal
herniae are associated.

41-A 15 month old boy presents with high fever of 6 days duration, rash, tender
neck glands, red eyes and cracked lips. Full term normal delivery with no
neonatal complications. Immunisations up to date. There was no family or social
history of note.
On examination temperature is 38.9C and he is miserable and clingy. Respiratory
rate is 30/min and heart rate 120/min, well perfused. The rash is maculopapular
erythematous and blanching. The hands and feet are swollen. He has tender
cervical gland enlargement, non-purulent conjunctivitis and prominent tongue
papillae.
What is the most likely diagnosis?
(Please select 1 option)

Acute Rheumatic fever

Henoch Schonlein disease


Kawasaki disease

Correct

Meningococcal sepsis
Stevens-Johnson Syndrome

The history is classical of Kawasaki disease (muco-cutaneous lymphnode


syndrome). The features described fulfil the international criteria, the diagnosis
being purely clinical. Often there is elevation of CRP, ESR and platelet count. IVIG
2g/kg should be given within the first 10d of illness to reduce the risk of coronary
artery aneurysm

42-A 2 month old baby is admitted with seizures and failure to thrive. Follwoing
a seizure he is found to have a blood glucose concentration of 1.2 mmol/L (3.06.0). Other biochemistry reveals a lactic acidosis and hyperlipidaemia.
Examination reveals hepatomegaly with palpable kidneys. What is the most likely
diagnosis?
(Please select 1 option)

Gaucher's disease
McArdle's disease
Niemann-Pick disease
Pompe Disease
Von Gierke disease

Correct

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

43-A 5-year-old girl presents with ataxia. She keeps falling to the left during a
ballet lesson. The symptoms persist, and parents take her to casualty. 41/40
gestation 2.9kg, with no neonatal problems.
On examination she is alert with temperature of 36.9C (tympanic), RR of 20/min
and HR of 95/min. She has obvious nystagmus, dysdiadochokinesis and falls to
the left when asked to walk.

What is the most likely diagnosis?


(Please select 1 option)

Brain tumour

Correct

Infections
Ingestions

The history is of subacute cerebellar abnormality in the absence of fever. The


most likely diagnosis is a posterior fossa tumour. This can be confirmed by CT or
MRI scan. The latter is preferable because the bony posterior fossa interferes with
CT images.

44-An 18 month old boy because of maternal concerns about delayed speech.
He was born at 39+4/40 weighing 2.6kg and there were no neonatal problems.
He sat at 12/12, and now pulls to stand. He is able to make a tower of 2 inchhigh bricks. He is saying 2-3 single words which his mother can understand and
seems to hear well. He drinks from a bottle. He is fully immunised, on no
medications, and there is no FH/SH of note.
On examination he looks well. He is on the 25th centile for height and weight and
OFC. There are no specific findings of note.
What is the most likely diagnosis?
(Please select 1 option)

Speech delay, isolated


Pervasive developmental disorder
Global developmental delay

Correct

Impaired hearing
Autism

The history suggests moderate global developmental delay. He is functioning at


about the 12 month level in gross motor, fine motor and vision, hearing and
speech, and social development. The history gives no indication of an underlying
cause for the delay. A detailed developmental assessment should be performed
plus investigations for an underlying cause. At minimum these should include
TFTs and karyotype to rule out Fragile X Syndrome. Other investigations may be
indicated if a specific disorder is suspected.

45-A 3-year-old child presents with delayed speech. Although he seems to


understand full sentences and commands he says only occasional single words.

He was born at 41/40 weighing 3.4kg and there were no neonatal problems. He is
fully immunised, on no medications, and comes from a middle class articulate
family.
On examination he is on the 25th centile for height, weight and OFC. There are
no specific abnormalities to find. ENT examination is unremarkable.
What is the most likely diagnosis?
(Please select 1 option)

Speech delay, isolated

Correct

Pervasive developmental disorder


Global developmental delay
Impaired hearing
Autism

The history is of isolated speech delay in a child who apparently hears normally.
Hearing should be formally checked, as it is the commonest reason for speech
delay.In this case the problem involves mainly expression rather than reception of
speech. Such speech problems may interfere significantly with schooling.

46-A 12-year-old boy complains of persistent clear nasal discharge during the
spring and summer. He constantly rubs his nose and his eyes. Sometimes he has
an associated cough. The symptoms usually get better in the autumn. He was
diagnosed with asthma at 2 years, but this has improved with age. He rarely
requires beta agonist inhaler.
On examination he has a clear nasal discharge, nasal speech, and pink nonpurulent conjunctivae.
What is the most likely diagnosis?
(Please select 1 option)

Gastroesophageal reflux
Allergic rhinitis

Correct

Sinusitis
Asthma
Croup

This atopic boy has classical symptoms of seasonal rhinitis. Although skin prick
testing may be helpful in resistant cases, it is usual to treat sufferers
symptomatically with oral antihistamines or topical steroids to eyes and nose.

47-year-old girl presents with high fever and severe left-sided throat pain. She
has had difficulty in swallowing over the last 2 days, and has been finding it
increasingly uncomfortable to open her mouth. Her voice is muffled and she
dribbles saliva. She was born at 41/40 gestation weighing 4.0kg and there were
no neonatal problems.
On examination she looks ill. Her temperature is 40.2C (tympanic), RR 15/min
and HR 100/min. ENT examination shows left tonsillar enlargement and exudate,
with a uvula deviated to the right.
What is the most likely diagnosis?
(Please select 1 option)

Retropharyngeal abscess
Foreign body aspiration
Anaphylaxis
Croup
Peritonsillar abscess

Correct

The history suggests a peritonsillar abscess (quinsy) on the left side, a


complication of Group A Strep. sore throat. A fever greater than 39.4C is
associated with severe disease, and treatment is by surgical drainage.

48-A 9-year-old boy presents with a history of headache and persistent green
nasal discharge. At night he has a cough and snores loudly. The headache is
exacerbated by leaning forwards.
On examination he is apyrexial, but has a persistent nasal obstruction and nasal
speech. He is tender over the maxillae and forehead.
What is the most likely diagnosis?
(Please select 1 option)

Gastroesophageal reflux
Allergic rhinitis
Sinusitis
Asthma
Croup

Correct

The picture is one of upper airways obstruction associated with nasal discharge,
most likely due to sinusitis. In this case the maxillary and frontal sinuses are
most likely to be involved

49-Which of the following statement is true of infections with Mycobacterium


tuberculosis:
(Please select 1 option)

Non-sputum producing patients are non-infectious

This is the correct answer

A positive tuberculin test indicates active disease


Lymph node positive disease requires longer treatment than pulmonary disease
Incorrect answer selected
In pregnant women treatment should not be given until after delivery
Pyrazinamide has high activity against active extracellular organisms

Only untreated smear positive pulmonary TB is likely to be infectious. Active


disease may be indicated by grade III/IV response to tuberculin. 80% of
individuals with history of BCG vaccination have grade I/II response. All forms of
pulmonary TB may be treated equally except tuberculous pleural effusion which
may require drainage (with large effusions causing breathlessness) and adjunct
corticosteroids to delay reaccumulation. Length of treatment for other forms are
bone TB 9 months, meningitis 1 year, drug resistance 2 years. Streptomycin has
high activity against extracellular organisms whilst pyrazinamide have high
activity against intracellular organisms.

50-A 41+5/40 gestation infant is born by SVD weighing 2.31kg with apgars of 7
at 1 minute and 9 at five minutes. Pregnancy was complicated by maternal
smoking and poor growth on antenatal ultrasound in the final trimester. There is
no family history of note.
On examination his weight is between the 0.4% and 3% and his OFC on the 25%.
He has peeling skin. His temperature on arrival in the neonatal unit is 35.6C and
BM stix is 2.0 mmol/l. There are no specific abnormalities to find.
What is the most likely diagnosis?
(Please select 1 option)

Asymptomatic hypoglycaemia
CMV infection
Edwards syndrome

Correct

Hypoparathyroidism
Sepsis

This child is small for gestational age, with preservation of head size. This
suggests late pregnancy malnutrition. As in this case, such children are at risk of
hypoglycaemia and hypothermia, as they have limited glycogen and brown fat
reserves. Hypothermia increases surfactant consumption, with a risk of
developing surfactant-deficient lung disease. Peeling skin indicates post-maturity.