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1-Theme:Substance abuse.

A Amnestic disorder
B Anxiety disorder

Hallucinogen persisting perception


disorder

D Intoxication
E

Intoxication delirium

Psychotic disorder

G Mood disorder
H Withdrawl
I Withdrawl delirium
For each scenario choose the most likely diagnosis:
A 14-year-old boy presents with deteriorating school performance. He seems withdrawn,
unenthusiastic and irritable.

Correct

This 14-year-old boy has features of depression - mood disorder - with a withdrawn
affect being typical.

A 15-year-old girl presents acutely with visual disturbance. She describes distressing images
of colourful objects with moving trails.

Correct

The 15-year-old girl has visual hallucinations, most likely due to "flashbacks" to
previous substance abuse. This may be associated with drug abuse. Psychotic
disorder is unlikely as visual hallucinations would not be expected.

A 13-year-old boy is brought to casualty, having been found at the side of the road. He
responds to voice but appears ataxic.

Correct

The 13-year-old boy is intoxicated. In children of any age with unexplained


neurological symptoms or signs, drugs or alcohol should be suspected.

Substance abuse is an increasing problem in schools, and may present to


educational, social, medical or law enforcement agencies. They may trigger
symptoms of mood or psychosis, and may be confused with these conditions.
Next question

2-A 17-year-old female presents after taking an overdose of her


grandmothers medication.
Investigations revealed a serum potassium of 6 mmol/L (3.5-4.9).
Which one of the following drugs is the most likely cause of this abnormality?
(Please select 1 option)
Aspirin
Digoxin

Incorrect answer selected

Fluoxetine
Omeprazole
Propranolol

This is the correct answer

Betablockers, particularly propranolol, in overdose can cause hyperkalaemia


by transmembrane shifts. It is useful to think of the opposite - beta-agonists
such as salbutamol are far more widely known to cause hypokalaemia.
Overdose of digoxin therapy most commonly manifests as rhythm
disturbance, and may be exacerbated by hyper- or hypokalaemia. Salicylate
overdose causes metabolic acidosis and respiratory alkalosis.
Next question

3-Theme:Investigation of Short stature.


A Bone age
B Chromosomal studies
C

Coeliac screen

D Cortisol profile

Growth hormone stimulation


tests
IGF-1

G Renal biochemistry
H Thyroid function tests
I Urinalysis
For each scenario choose the most important
A 3-year-old girl presents with short stature. She is otherwise in good health. She is on the
25th centile for weight, less than 0.2% for height and has normal body proportions.

Incorrect - The correct answer is Growth hormone stimulation tests

The 3-year-old girl appears normal but short, with preserved weight. She is unlikely
to have a nutritional problem, but probably has some form of growth hormone
deficiency. There is nothing in the history to suggest hypopitutarism. GH stimulation
testing is most important.

A 4-year-old boy presents with short stature and obesity. He has mild learning difficulties.

Incorrect - The correct answer is Chromosomal studies

The 4-year-old boy has Prader-Willi which is best confirmed by karyotype. This
should be distinguished from Laurence-Moon-Biedl, where there is associated
polydactyly or syndactyly and retinitis pigmentosa.

A 6-year-old boy presents with short stature. He is on the 25% for height and below the 2nd
centile for weight, and appears pale.

Correct

The 6-year-old boy has preserved height but low weight plus pallor. Malabsorbtion is
therefore likely, and a coeliac screen should be performed.

4-Theme:Neck mass.
A Branchial cyst
B Cystic hygroma
C

Dermoid cyst

D Goitre
E

Haemangioma

Laryngocoele

Sternomastoid
tumour

H Teratoma
I Thyroglossal duct cyst
For each scenario choose the most likely diagnosis:

A 4-year-old boy presents with a red tender swelling in the midline, which rises on swallowing.

Correct

The 4-year-old boy has a midline mass. The differential lies between thryoglossal
cyst, goitre, dermoid cyst, teratoma or laryngocoele. The clinical presentation
suggests an infected thyroglossal cyst.

A 2 day old infant presents with large transilluminating mass in the right posterior triangle of
the neck.

Correct

The 2 day old infant has mass in the posterior triangle which transilluminates. A
cystic hygroma is likely.

A 2 week old infant is noted to keep her head to the left. On examination she has a palpable
lump in the left side of the neck.

Correct

The 2 week old infant has a firm mass in the body of sternocleidomastoid associated
with decreased head movement to the contralateral side, a sternomastoid tumour.
This responds to stretching exercises.

Neck masses may be congenital or acquired. Congenital masses may be in the


midline, in the anterior triangle, posterior triangle, body or sternocleidomastoid or
anywhere. Masses in the anterior triangle represent branchial cleft cysts.
Haemangiomas can occur anywhere, but are easily identified by their soft, spongy
red characteristics.

On examination of neck swellings...

5-Theme:Abdominal pain.
A Abdominal migraine
B Familial Mediterranean fever

Gastro-oesophageal reflux

D Giardiasis
E

Inflammatory bowel disease

Meckel's diverticulum

G Pancreatitis
H Peptic ulcer disease

Recurrent abdominal pain of

childhood
For each scenario choose the most likely diagnosis:

A 9 month old boy presents with abdominal pain and black offensive stools. On examination he
appears pale.

Correct

In the 9 month old boy the major symptom is melaena. The pallor suggests
significant bleeding. At this age the most likely diagnosis is Meckel's diverticulum.

A 12-year-old girl presents with a 1 year history of central abdominal pain lasting 1-3 hours at
a time.

Correct

The 12-year-old girl suggests recurrent abdominal pain of childhood . In abdominal


migraine there may be specific precipitants, and associated headache and a family
history.

A 3-year-old boy presents with a 3 day history of fever, abdominal pain and pain and swelling
of knees and ankles. He has had two similar episodes in the past. On examination he has a
3cm liver.

Incorrect - The correct answer is Familial Mediterranean fever

The 3-year-old boy has recurrent episodes of abdominal pain, fever and arthritis.
There is hepatomegaly. All these point to Familial Mediterranean Fever.

6-Theme:Connective tissue disorders.


A Camurati-Engelmann syndrome
B Ehler-Danlos syndrome
C

Beals syndrome
Fibrodysplasia ossificans
congenita

Homocystinuria

Marfan syndrome

G Osteogenesis imperfecta type 1


H Osteogenesis imperfecta type 2
I Osteogenesis imperfecta type 4
For each scenario choose the most likely diagnosis:
A 9-year-old girl has learning difficulties with lens subluxation, kyphosis and malar flush.

Incorrect - The correct answer is Homocystinuria

Marfan should be distinguished from Homocystinuria, which is associated with


learning difficulties, and is the diagnosis in the 9-year-old girl. The lens subluxes in
the opposite direction too.

A 9-year-old boy is referred with joint hypermobility, fragile skin and easy bruising.

Incorrect - The correct answer is Ehler-Danlos syndrome

The 9-year-old boy is characteristic of Ehlers-Danlos. The complex genetics of this


spectrum of disorders is gradually being revealed. Clinical appearances are very
variable.

A 12-year-old girl presents with kyphosis. She is on the 90th percentile for height, and has
long fingers, pectus excavatum and a systolic murmur.

Correct

The 12-year-old girl is characteristic of Marfan syndrome . The aortic media gradually
stretches, resulting in aortic dissection in later age. Before this, valvular leaks e.g.
floppy mitral valve may be present. There may be a high-arched palate and positive
Steenburg sign, where the thumb folded in the palm protrudes beyond the other side
of the palm.

Connective tissue syndromes represents a spectrum of disorders from mild to very


severe. Joints and skin are particularly affected, but any mesoderm-derived tissue
may be.
Next question

7-Warfarin metabolism may be inhibited by


True / False
Oral contraceptive
Omeprazole
Aspirin

Correct

Incorrect answer selected

Correct

Rifampicin

Incorrect answer selected

Metronidazole

Incorrect answer selected

The anti-coagulant effects of warfarin may be exacerbated through the inhibition of


its metabolism by cytoP450. Omeprazole, Metronidazole, cimetidine and amiodarone
may all do this and so INR should be carefully monitored. However, Rifampicin
induces P450 and so would reduce the anticoagulant effects of Warfarin (as may
other inducers of P450 such as some anticonvulsants). Oestrogen may reduce the
anticoagulant effect independently of P450.

Detailed list of drug interactions...


Next question

8-Which of the following antibiotics inhibit microbial protein synthesis?


True / False
Vancomycin

Correct

Chloramphenicol
Ampicillin

Correct

Correct

Tetracycline

Correct

Gentamicin

Incorrect answer selected

Antibiotic action can be categorised into:

inhibition of cell membrane synthesis: Lincomycins


inhibition of protein synthesis: tetracyclines, macrolides, aminoglycosides,
chloramphenicol
inhibtion of DNA formation: sulphonamides/trimethoprim, quinolones,
metronidazole
inhibition of cell wall synthesis: penicillins, vancomycin, cephalosporins,
macrolides
Next question

9-A moderately dehydrated baby with acute gastroenteritis:


True / False
Has a fluid deficit of about 100ml per kilogram

Incorrect answer selected

Is more likely to have hypernatraemia than hyponatraemia


Will have loss of skin turgor

Correct

Correct

If breast-fed, should stop oral feeding

Correct

If formula-fed will need a lactose-free milk on commencing oral feeding

Incorrect

answer selected

A moderately dehydrated baby has a loss of 6-9% of body weight and a fluid
deficit of around 100mls/kg.
Hyponatraemia or a normal serum sodium is more likely to be present than
hypernatraemia.
Decreased skin tone and tissue turgor will be present, also oliguria, dry
mucous membranes and sunken fontanelle. Breast feeding should be
continued and supplement oral feeds may be required to maintain adequate
volumes. Formula fed infants should not have their formula changed and milk
feeding should not be discontinued.

10-In infantile hypertrophic pyloric stenosis:


True / False
Boys outnumber girls by 4:1

Correct

Vomiting typically starts at 2 to 4 weeks


Vomitus is bile-stained
Babies refuse to suck

Correct

Correct
Correct

There is often a hypochloraemic hypokalaemic alkalosis

Correct

The male to female ratio is 4:1. The clinical symptoms commonly appear in
this time frame. The vomiting is not bile-stained but consists of large
volumes of curdled milk. Appetite, and feeding are normal or increased. The
characteristic metabolic abnormality is hypochloraemic hypokalaemic
alkalosis.
Actual definition of pyloric stenosis...
Next question

11-The following are characteristic features of hereditary spherocytosis:


True / False

It is transmitted as an autosomal dominant trait


It does not present during the neonatal period

Correct
Correct

In a 5-year-old child, the spleen is likely to be enlarged


The Coombes test is usually positive

Correct

Correct

The majority of those affected will die before reaching adult life

Correct

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 2 conditions. Splenectomy before
the age of 5 stops further haemolysis and the patient can lead a normal life.
Next question

12-In a patient with a haemolytic anaemia, e.g. hereditary spherocytosis:


True / False
The level of bilirubin (normal less than 17mmol/l) is likely to be between 40100mmol/l

Correct

The bilirubin will be fat soluble

Correct

The diagnosis will be confirmed by the presence of bilirubin in the urine


the patient may have an increased reticulocyte count

Correct

Correct

Therapy with iron, e.g. Ferrous Sulphate solution is indicated to correct the
anaemia

Correct

The bilirubin is likely to be raised secondary to chronic haemolysis. The


bilirubin will be unconjugated and therefore fat soluble. The diagnosis is
confirmed by an osmotic fragility test which causes cell lysis. Increased
reticulocyte count results from haemolysis. The treatment is splenectomy to
prevent destruction of red cells by the spleen and restore red cell survival.
Read more about Haemolytic anaemia on e-Medicine...

13-Haemophilia A:
True / False
Is associated with a factor VIII level of 0-1% in severe cases

Incorrect answer

selected
Causes severe and permanent arthritic changes

Correct

Usually causes bleeding from the umbilicus at birth

Correct

Is associated with an increased risk of serum hepatitis


May be managed in the home

Correct

Incorrect answer selected

Mild cases are associated with a factor VIII level of >5%,moderate 1-4% and
severe <1% . Haemarthrosis causes chronic deformities. Bleeding from the
muscles and joints results , rather than from the skin and umbilicus. Multiple
transfusions of blood products have lead to the development of transfusion
related hepatitis and HIV in many affected patients. The patients may be
managed at home with self administration of recombinant factor VIII therapy
and aggressive haemostasis.
What exactly is Haemophilia A?...
Next question

14-A 12-year-old girl is diagnosed with a duodenal ulcer. Which of the following drugs is it
reasonable to consider for treatment:
True / False
Dexamethasone
Omeprazole
Ranitidine

Correct

Correct
Correct

Calcium buffers
Sodium Salicylate

Incorrect answer selected


Correct

Antacids containing magnesium, calcium and aluminium may be used to neutralise


excess acids. Acid secretion may be inhibited with H2 blockers such as Omeprazole
and Ranitidine.Sucralfate may also be used to coat the ulcers locally. Products that
enhance ulcer development such as steroids and NSAIDS should be avoided.

What are peptic ulcers and how are they treated?...

15-Treatment of salicylate poisoning may include:


True / False
Encouraging a high fluid intake
Forced acid diuresis

Correct

Vitamin K administration
Gastric lavage

Correct

Correct

This is the correct answer

Sodium bicarbonate

Correct

Treatment of children with Salicylate poisoning includes ABC as required. Gastric


lavage is required. Blood levels are often misleading especially if enteric coated
tablets have been taken. It is important to monitor the urine volume and ph at all
times. A forced alkaline diuresis is induced with a bicarbonate infusion. Correction of
dehydration and maintenance is calculated with haemodialysis in severe cases.In the
event of a coagulopathy Vitamin K administration may be necessary.
Next question

16-Sodium Valproate:
True / False
Is effective in treating petit mal

Correct

Often causes hyperactivity in children


May prolong the bleeding time

Correct

Correct

Levels above the therapeutic range may be tolerated without side effects
Incorrect answer selected
Is the drug of choice in controlling epilepsy in pregnancy

Incorrect answer

selected

Sodium valproate is used for the treatment of all forms of epilepsy including
absences. Hyperactivity and behavioural problems are listed in recognised side
effects however are very rarely seen. Plasma valproate levels are unhelpful.There
may be dose related side effects and levels above the therapeutic index may be
tolerated without symptoms. There is a potential for prolonged bleeding as valproate
can cause thrombocytopaenia as well as inhibition of platelet aggregation.Liver
dysfunction may also occur.There is a risk of teratogenic side effects with anti

convulsant therapy and there is a particular association of neural tube defects with
the use of sodium valproate.Patients should be counselled and informed and the
treatment changed (Lamotrigine is an alternative).

17-Calcitonin:
True / False
is a polypeptide hormone

Correct

is secreted by the thyroid epithelial cells

Incorrect answer selected

secretion is increased by a fall in serum calcium concentration


inhibits bone resorption

Correct

Correct

is secreted in excess in patients with medullary carcinoma of the thyroid.


Incorrect answer selected

Cacitonin is a peptide hormone released from the C cels of the thyroid in response to
hypercalcaemia. It's hypocalcaemic effects are mediated by preventing bone
resorption by osteoclasts.
Next question

18-Theme:Paediatric accident and emergency


A 3
B 4
C

D 6
E

G 9

1
0

1
1
1

2
Choose the Glasgow coma Scale score for the following
A 3-year-old boy was hit by a car. He opens his eyes to voice, cries to pain, and localises to
pain.

Incorrect - The correct answer is 11

Eyes 3, verbal 3, motor 5

A 7-year-old girl was an unrestrained passenger in a car accident. She does not open her
eyes, has incomprehensible sounds, and flexes to pain.

Incorrect - The correct answer is 6

Eyes 1, verbal 2, motor 3

A 2-year-old girl fell off a fairground ride. She opens her eyes to pain, has consolable crying
and withdrawls to pain.

Correct

Eyes 2, verbal 4, motor 4

Glasgow Coma Scale (4-15 years)


EYE OPENING spontaneously 4
To verbal stimuli 3
To pain 2

No response 1

BEST MOTOR RESPONSE


Obeys verbal response 6
Localises to pain 5
Withdraws from pain 4
Abnormal flexion (decorticate) 3
Abnormal extension (decerebrate) 2
No response 1

BEST VERBAL RESPONSE


Orientated and converses 5
Disorientated and converses 4
Inappropriate words 3
Incomprehensible sounds 2
No response 1

Childs Glasgow Coma Scale (<4yrs)

EYE OPENING
Spontaneous 4
To verbal stimuli 3
To pain 2

No response 1

BEST MOTOR RESPONSE


Spontaneous/obeys verbal commands 6
Localises to pain or withdraws from touch5
Withdraws from pain 4
Abnormal flexion to pain 3
Abnormal extension to pain 2
No response 1

BEST VERBAL RESPONSE


Alert, babbles, coos as usual 5
Less than usual words, spontaneous irritable cry 4
Cries only to pain 3
Moans to pain 2
No response 1

The GCS is always the best possible response. The younger children are
difficult, and the modified Children's score is used.
Next question

19-Theme:Conduct disorders.
A Adjustment disorder with conduct disturbance

Adjustment disorder with emotional


disturbance
Attention deficit disorder

D Attention deficit hyperactivity disorder


E

Conduct disorder confined to family context

Depressive conduct disorder

G Hyperkinetic conduct disorder


H Oppositional defiant disorder
I Unsocialised conduct disorder
For each scenario select the most likely diagnosis:
A 4-year-old boy is referred with temper tantrums. He often loses his temper, argues with
adults, and deliberately annoys people, blaming others for mistakes.

Incorrect - The correct answer is Oppositional defiant disorder

A 5-year-old boy is referred with aggression. At home he bullies other children, picks fights,
and breaks toys deliberately.

Correct

A 6-year-old boy is referred with a history of hitting other children at home and at school. His
mother died 3 months ago.

Incorrect - The correct answer is Adjustment disorder with conduct disturbance

Conduct disorders are those associated with aggressive or antisocial


behaviour.

In the 4-year-old boy the behaviour is generalised oppositional defiance.


In the 5-year-old it is specific to the home situation.
In the 6-year-old it is a reaction to bereavement.
Next question

20-Theme:Somatisation.
A Dissociative motor disorder

Dissociative anaesthesia and sensory


loss
Factitious disorder

D Hypochondiacal disorder
E

Hysterical conversion syndrome

Neurasthenia

G Persistent somatoform pain disorder


H Somatoform autonomic dysfunction
I Somatisation disorder
For each scenario choose the most likely diagnosis:

A 15-year-old girl presents with left leg paralysis and sudden onset of blindness in the right
eye. On examination there are no neurological abnormalities.

Correct

The 15-year-old girl has hysterical conversion symptoms. These are bizarre, fit no
known physical pattern, and often the child seems rather blase about apparently
severe symptoms. They often appear or disappear at key moments (eg term time,
holidays). Over-investigation may exacerbate them.

A 12-year-old girl is referred with a 2 year history of recurrent abdominal pain. The pain is
periumbilical without associated features.

Incorrect - The correct answer is Persistent somatoform pain disorder

The 12-year-old girl has recurrent abdominal pain, which affects 10% of children at
some point. It is associated with normal growth and development.

A 14-year-old boy has had recurrent headaches associated with nausea for 1 year. It feels like
a dull frontal ache and is worse when he is tired.

Incorrect - The correct answer is Persistent somatoform pain disorder

The 14-year-old boy has tension headaches. A pain diary may identify avoidable
causes. Treatment is with paracetamol. It forms part of a spectrum with recurrent
abdominal pain and cyclical vomiting.

Somatisation refers to those disorders where psychological problems manifest as


physical symptoms.Review article

21-Theme:Learning disorders.
A

Acquired aphasia with epilepsy

Expressive language disorder

Mild mental retardation

Moderate mental retardation

Receptive language disorder

Specific reading disorder

Specific spelling disorder

Specific disorder of arithmetic skills

I
Specific speech articulation disorder
For each scenario select the most likely diagnosis:
A 5-year-old boy is referred by the school because of poor progress in all areas. No specific
physical abnormality is found.

Incorrect - The correct answer is Moderate mental retardation

The 5-year-old boy has moderate mental retardation. If the defect was mild, then
presentation may be delayed until 7 or 8 years when more complex topics are
covered.
A 5-year-old girl is referred with reading difficulty. Otherwise her school progress is
satisfactory.

Correct

The 5-year-old girl has specific reading disorder.


A 6-year-old boy is referred with difficulty in comprehending speech.

Correct

The 6-year-old boy has a receptive language disorder. His hearing should be
checked, as he may have glue ear.
Learning disorders may be generalised or specific. Specific reading disorder
(dyslexia) can be due to difficulties with vision, comprehending letter shapes,
comprehending syntax, or a combination. Speech disorders can be due to problems
with reception, comprehension or expression.
Next question

22-heme:Sick young children


A

Blood cultures

Blood glucose

Blood PCR for meningococcus

Clotting screen

CT scan head

Fundoscopy

Lumbar puncture

Urine metabolic screen

I
USS head
For each scenario choose the most informative initial diagnostic test:

A 3-year-old boy presents with a 24 hour history of fever and decreasing responsiveness. On
examination he is alert and has slight neck stiffness.

Correct

The 3-year-old boy has signs of mild meningeal irritation, but no focal neurology nor
decreased level of consciousness. An LP is therefore safe and will be most
informative test.
A 3 month old boy presents with a history of poor feeding and twitching of all 4 limbs for 3
minutes. On examination his temperature is 35.4oC, he has cool peripheries and he responds
to pain. His fontanelle feels full. He has 3 1 cm bruises on either side of his chest.

Incorrect - The correct answer is Fundoscopy

The 3 month old boy has shaken baby syndrome. This grip marks are highly
suggestive. Although USS head can be helpful subdurals may be missed. CT scan will
be helpful, but delayed. Fundoscopy showing flame-shaped haemorrhages will give
direct and rapid evidence of the diagnosis.
A 4 month old girl presents with history of fever and poor feeding. On examination she has a
petechial rash over the trunk and is responding to pain.

Incorrect - The correct answer is Blood cultures

The 4 month old girl has meningococcal septicaemia until proven otherwise. As the
child has a decreased LOC and LP should probably be deferred. Blood cultures should
be taken and antibiotic treatment commenced. PCR has excellent sensitivity and
specificity, but in most of the UK goes to reference labs and the results are not
available quickly.
Next question

23-Theme:Wheeze
A

Aspiration

Bronchiolitis

Bronchospasm

Foreign body

Hilar adenopathy

Pneumonia, Mycoplasma

Pneumonia, Chlamydia

Pulmonary oedema, cardiogenic

I
Pulmonary oedema, non-cardiogenic
For each scenario choose the most likely diagnosis:
A 2-year-old child presents with a 5 day history of fever and blanching rash. She develops red
eyes and sore lips. 2 days later she becomes breathless and wheezy.

Incorrect - The correct answer is Pulmonary oedema, cardiogenic


A 4-year-old child presents with a 3 month history of progressive unwellness. He has large
rubbery glands in the neck and audible wheeze.

Correct
A 13-year-old girl presents with a 5d history of myalgia, fever, and wheeze. She has never had
chest problems before.

Incorrect - The correct answer is Pneumonia, Mycoplasma

Not all that wheezes is asthma!


The 2-year-old child has Kawasaki disease and heart failure, an uncommon
cause of wheeze at this age.
The 4-year-old has a malignancy with external airway compression from
mediastinal glands.
The 13-year-old is an adolescent with atypical pneumonia, most likely
Mycoplasma. Mycoplasma and Chlamydia are the exceptions to the rule that
wheezing does not respond to antibiotics.
Next question

24-Theme:Vaginal discharge.
A

Allergic

Bacterial vaginosis

Child sex abuse

Foreign body

Pinworms

Rectovaginal fistula

Tumour

Vulvovaginitis, infectious

I
Vulvovaginitis, non-specific
For each scenario choose the most likely diagnosis:
A 4-year-old girl presents with persistent scratching of her anus. Perineal examination is
unremarkable.

Correct

Perianal itching is usually due to pinworms (threadworms). This can be confirmed by


a sellotape test, where a strip of sellotape is placed at the anal margin on waking
and examined for worms.
A 4-year-old girl presents with vaginal irritation and scanty discharge. On examination she has
minimal perineal redness.

Incorrect - The correct answer is Vulvovaginitis, non-specific

Vaginal irritation with redness is common as girls learn to wipe themselves after
defaecation and as the skin is thin and sensitive at this age. Avoidance of occlusion
(eg plastic pants), irritants (eg bubble bath) and keeping the perineum dry usually
results in rapid resolution.
A 9-year-old girl presents with vaginal irritation and offensive discharge. On examination a
mucopurulent discharge is seen.

Correct

Offensive mucopurulent discharge suggests a significant infection, such as chlamydia


or gonorrhoea. Swabs should be taken, including for these organisms. If positive
then child sex abuse has been confirmed.
Next question

25-Chronic stridor
A

Angioedema

Croup

Epiglottitis

Laryngeal cleft

Laryngomalacia

Mass lesion

Papillomatosis

Retropharyngeal abscess

Vascular ring

J Vocal cord paralysis


For each scenario choose the most likely diagnosis:
A 6 week old boy presents with an inspiratory noise. This has been present since birth, but has
worsened since he picked up the family cold.

Correct

The presentation of the 6 week old is highly suggestive of laryngomalacia. This is


seldom severe, but may worsen during intercurrent infections. It improves
spontaneously and seldom requires surgery.
A 3 month old boy presents chronic stridor with seal-bark cough. He has previously had
pneumonia. On examination he has severe respiratory distress.

Incorrect - The correct answer is Vascular ring

The 3 month old has chronic stridor, a barking cough and previous pneumonia. This
points to a vascular ring. Other presentations can resemble asthma. They can also
present with recurrent pneumonia or cyanotic spells.
A 5 month old girl presents with chronic stridor. Her head circumference is on the 97%, having
been on the 50% at birth.

Correct

The 5 month old girl has associated an enlarging head circumference, which points to
hydrocephalus. A vocal cord palsy is therefore most likely.
Chronic stridor suggests a structural, neurological or chronic acquired lesion.
Next question

26-Chest signs that are reliable diagnostically are found in:

True / False

A neonate with meconium aspiration

Correct

A 3-month-old child following aspiration pneumonia

Correct

A 4-month-old child with bronchiolitis

Incorrect answer selected

A 6-month-old with lobar pneumonia

Incorrect answer selected

A 2-year-old with inhaled foreign body

Incorrect answer selected

Unfortunately, clinical chest signs are remarkably unhelpful, and in general


are less helpful the younger the child.
Although in meconium aspiration chest signs may be found such as coarse
crepitations, the diagnosis is usually on the basis of the general appearance
of the child (meconium stained, meconium in the oro-phayrnx or recovered
from the lungs) associated with an appropriate chest x ray.
A 3-month-old with aspiration pneumonia normally presents with a sudden
onset of respiratory distress, but focal signs are often difficult to locate at
this age.
A child with bronchiolitis, however, has more generalised signs, and a fairly
typical clinical picture.
Lobar pneumonia can easily be missed in a 6-month-old child, and a chest x
ray is, therefore, usually included as part of a septic screen.
The diagnosis of inhaled foreign body may be difficult even on x ray in a 2year-old child.

27-Theme:Investigation of wheeze
A Allergy testing
B Barium swallow
C

Ciliary motility

D CT scan chest
E

Exercise test

Fluoroscopy
Methocholine
challenge

H pH studies
I

Sweat test

For each scenario choose the most specific diagnostic test.


A 3-week-old infant presents with a history of increasing breathlessness since birth. There is
decreased air entry over the right base.

Correct

This is a child with progressive breathlessness and focal decreased air entry.
This is likely to be due to a mass lesion, such as cystic adenomatous
malformation. The chest x ray may confirm this, but a CT scan of chest will
be definitive.
A 3-month-old boy presents with recurrent chestiness and wheeze. He was born at term weighing
3.7 kg and is now 4.3 kg.

Incorrect - The correct answer is Sweat test

This boy has associated failure to thrive, so cystic fibrosis should be


excluded. In older children with sinus/ear infections -/+ situs inversus, ciliary
motility disorders should be considered.
A 15-month-old boy presents with sudden onset of coughing, lacrimation and wheeze. He has
previously been completely well.

Correct

This child's presentation is associated with choking suggestive of foreign


body aspiration. Fluoroscopy or microlaryngobronchoscopy are most helpful
here, as many inhaled objects are not radio-opaque and fail to show up on
standard chest x ray.

Wheeze may be acute or chronic. Acute wheeze may be associated with


infection or foreign body. Chronic wheeze may be associated with a normal or
abnormal chest x ray

28-Useful measurements to distinguish between acute renal failure and


chronic renal failure include:
True / False

x Ray of the wrist

Correct

Ultrasound scan of the kidneys


Serum phosphate level
Anthropometry

Correct

Correct

Correct

Serum potassium

Correct

In acute renal failure, there may be metabolic acidosis with


hyperphosphataemia and hyperkalaemia. The kidneys may be swollen, for
example with renal vein thrombosis. Because of the acute onset, there is
unlikely to be any changes on the x ray and growth failure will be absent.
In chronic renal failure, there may be evidence of renal osteodystrophy on x
ray, with growth failure evident on anthropometry. Ultrasound scan may
show a shrunken scarred kidney with evidence of structural malformation.
Serum potassium and phosphate, as in the acute case, are often elevated,
and associated with acidosis, and secondary hyperparathyroidism.
Copyright 2002 Dr Colin Melville

29-Theme:Mode of inheritance
A

Autosomal codominant

B Autosomal dominant
C

Autosomal recessive

D Polygenic

Single gene defect

X-linked dominant

G X-linked recessive

Select the most likely mode of inheritance for the following patients'
conditions.
A 17-year-old female developed insulin dependent diabetes mellitus. Her uncle and grandmother
also had diabetes mellitus.

Correct

There is no specific mode of inheritance associated with diabetes mellitus.


The inheritance is therefore considered polygenic - many genes contributing
rather than one single gene defect. There are a few rare exceptions such as
the DIDMOAD syndrome (diabetes insipidus, diabetes mellitus, optic atrophy
and nerve deafness) (Wolfram syndrome) which is an autosomal recessive
condition.
A 4-year-old male child presents with mild jaundice, pallor and a palpable spleen tip. His father has
had a work up for anaemia.

Correct

This child has hereditary spherocytosis, which has an autosomal dominant


inheritance pattern.
A 14-year-old boy presents with increasing instability, ataxia and tremor with recurrent respiratory
and sinus infections. On examination he has prominent capillaries on his sclerae.

Correct

This male has the typical features of ataxia telangiectasia. It is a rare


disorder of childhood that occurs in about 1/40,000 and 1/100,000 persons
worldwide.
The ailment is progressive. By their teens, patients with AT are frequently
wheelchair-bound.

A 4-year-old boy developed swelling of his left knee with trivial injury. He had similar episodes like
this before. His grandfather had the same disease.

Correct

This boy's case is typical of haemophilia A or possibly B (Christmas disease).


Haemophilia A often presents at a young age with spontaneous bleeding into
a joint. The level of factor VIII (A) or IX (B) in the blood determines the
severity of haemophilia. Factor levels of less than 1-2% are found in subjects
with severe haemophilia, and they experience spontaneous bleeds into joints
or muscles.
Both are X-linked recessive inheritance.
An 18-year-old female underwent caries tooth extraction and developed profuse bleeding. On
history she revealed menorrhagia. Her mother and her grandfather had the same disease.

Correct

This young woman with a bleeding tendency following a dental procedure,


together with menorrhagia, suggests von Willebrand disease. This disease is
due to a deficiency of von Willebrand factor and is inherited in an autosomal
dominant pattern with variable penetrance most commonly, but rarely can be
recessive.
von Willebrand's disease is the most common hereditary bleeding disorder. It
affects both sexes approximately equally. Most cases are mild, and bleeding
may occur after a surgical procedure/tooth extraction. It is also associated
with menorrhagia.

30-A 16-year-old female is admitted after taking an overdose of her


mother's propranolol tablets approximately two hours ago.
On examination she is drowsy and has a pulse of 40 beats per minute with a
blood pressure of 80/40 mmHg. She is treated with activated charcoal, IV
fluids and IV atropine but her bradycardia and hypotension fail to respond.
Which of the following would be the most appropriate next stage in her
management?

(Please select 1 option)

IV adenaline

Incorrect answer selected

IV amiodarone
IV glucagon

This is the correct answer

IV phenytoin
Insertion of temporary pacemaker

In those in whom initial atropine is unsuccessful, IV glucagon is a


recommended treatment for beta-blocker overdose, with some evidence
indicating improvement in bradycardia and blood pressure

31-Which of the following investigations is most likely to detect the earliest


changes of Perthes' disease?
(Please select 1 option)

Plain x ray
CT
MRI
Ultrasound
Nuclear scan

Incorrect answer selected


This is the correct answer

Perthes' disease is the name given to idiopathic avascular osteonecrosis of


the capital femoral epiphysis of the femoral head.
Plain x rays of the hip are extremely useful in establishing the diagnosis, but
Technetium 99 bone scan is helpful in delineating the extent of avascular
changes before they are evident on plain radiographs.
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32-A 17-year-old girl was found collapsed and drowsy. Her 12-lead ECG
showed a sinus tachycardia of 120 beats per minute with a corrected QT
interval of 500 ms (normal <470). Which of the following drugs is the most
likely cause of her presentation?

(Please select 1 option)

Amphetamine

Incorrect answer selected

Diphenhydramine

This is the correct answer

Glue sniffing
Methadone
Methanol

You are given here the option of choosing between two drugs that can
possibly cause prolongation of QT - diphenhydramine and methadone.
However, bearing in mind that she has collapsed and drowsy with a
tachycardia, the most likely option is diphenhydramine as the tachycardia is
more typical. Many drugs can cause a prolonged QT interval.

33-A 16-year-old girl presents with bilateral cervical lymphadenopathy. Her


lymph node biopsy reveals a nodular sclerosing Hodgkin's disease.
Which one of the following features indicates a poorer prognosis?
(Please select 1 option)

Fatigue
Mediastinal mass of 3cm
Night sweats

Correct

Pruritis
Recent Epstein-Barr virus infection

Important prognostic features in Hodgkin's disease (HD) are Stage B


symptoms
- Fever
- Night sweats
- Weight loss
A mass of > 10 cm in size is alos a poor prognostic factor.
Therefore although fatigue and pruritus common, have no prognostic
significance.

EBV infection commonly associated with HD but has no prognostic


significance.

34-A girl of 6 attending a normal school is referred to the clinic because she
is short.
Her height is 4 cm below the third centile for age and her weight 1 kg below
the third centile. Her bone age is 4.2 years. Her mother's and father's
heights are on the 25 and 50 centiles respectively.
th

th

Which one of the following statements correctly applies?


(Please select 1 option)

A thyroid profile should immediately be requested


Findings of poorly felt femoral pulses suggest that chromosome analysis might be required
This is the correct answer
From these measurements the child is failing to grow
Physical measurements are compatible with growth hormone deficiency
Physiological (constitutional) short stature is unlikely

Incorrect answer selected

A history of delayed growth and puberty in the family should be elicited. One
single measurement of growth is not sufficient to indicate growth hormone
deficiency or thyroid disease.
Poorly felt femoral pulses suggest coarctation and Turner's.
Constitutional short stature is the most commonly encountered reason.
Another growth measurement is required to assess growth velocity.

35-A 4 day old girl is admitted with jaundice, which was first noted the
previous day. The pregnancy had been normal. She was born at 37/40
gestation weighing 3.29kg and there were no neonatal problems. No family
or social history of note.
On examination her temperature is 36.8C, respiratory rate 32/min and pulse
110/min. Her current weight is 3.10kg. Moderate jaundice over face and
trunk. There is no liver enlargement and her stools are pigmented.
What is the most likely diagnosis?
(Please select 1 option)

ABO incompatibility
Breast milk jaundice
Hypothyroidism
Physiological jaundice

Correct

Rhesus disease

The history is of jaundice beginning on day 3 in an otherwise healthy child.


This suggests physiological jaundice. The degree of jaundice is assessed by
serum SBR, and phototherapy may be required if it exceeds the treatment
threshold. A urine dipstick should be performed to exclude occult UTI.

36-A 2-year-old boy is seen in the Emergency Department 30 minutes after


ingesting 150 ml of amitriptyline syrup. He is fully conscious.
Which of the following is/are true?
True / False

An urgent plasma amitriptyline estimation is essential


Cardiac arrhythmias should be anticipated

Correct

Correct

He can go home after two hours observation provided he remains well


The pupils are likely to become constricted
Vomiting should be induced

Correct

Incorrect answer selected

Correct

This is the commonest agent responsible in paediatric cases of fatal


poisoning.
Features at presentation include drowsiness or agitation in mild cases. Other
CNS manifestations include

seizures

coma

myoclonic twitches

choreoathetosis

pyramidal tract signs.

Patients may present with blurred vision and mydriasis.

Arrhythmias are common; the most common lethal arrhythmia being


ventricular arrhythmia. Urinary retention and constipation may also be
present.
Treatment involves ABC resuscitation as necessary; gastric lavage followed
by activated charcoal in severe poisoning, and treatment of the arrhythmias
and seizures as necessary.
Even in mild cases it is important to monitor patients carefully.
Further reading:
Amitriptyline NetDoctor

37-Theme:Recurrent infection
A Ataxia-telangiectasia
B Chdiak-Higashi
C

Chronic granulomatous disease

D Common variable immunodeficiency


E

DiGeorge syndrome

Leukocyte adhesion deficiency

Severe combined immune


deficiency

H Wiskott-Aldrich syndrome
I

X-linked agammaglobulinaemia

For each scenario choose the most likely diagnosis.


A 4-year-old boy presents with cough and fever. Chest x ray shows multiple cystic areas in the left
lung. He was admitted for drainage of three skin abscesses over the previous two years.

Incorrect - The correct answer is Chronic granulomatous disease

This child with recurrent abscesses who develops findings compatible with
staphylococcal pneumonia. Recurrent infections with catalase positive
organisms is characteristic of chronic granulomatous disease. He has no
cutaneous stigmata of Chdiak-Higashi (silvery hair, photophobia).
A 5-month-old girl presents with failure to thrive (FTT) and chronic diarrhoea. Chest x ray shows
consolidation in the right middle lobe and left base.

Correct

This scenario fits best with severe combined immunodeficiency. In such


cases, the absolute lymphocyte count is almost always <1 x 10 /l.
9

A 5-month-old boy presents with cough and fever. On examination he has chronic eczema with
excoriations and petechiae. Chest x ray shows patchy changes on both sides.

Correct

This child has symptoms highly suggestive of Wiskott-Aldrich syndrome.


In assessing the child with recurrent infections it is first important to look at
general health, as normal children get 6-12 infections per year.
Secondly consider atopy, especially if recurrent chestiness is the problem.
An unwell child, growing poorly with recurrent proven infections deserves
detailed evaluation.

38-Which of the following is a form of generalised seizure?


(Please select 1 option)

Automatisms
Aversive seizures
Benign rolandic epilepsy
Epilepsia partialis continua
Lennox-Gastaut syndrome

Correct

Seizures may be classified as:

a) Partial

Simple partial (consciousness retained), motor,


sensory, autonomic, psychic

Complex partial (consciousness impaired)

Simple partial followed by impaired consciousness,


or consciousness impaired at onset

Partial seizures with secondary generalisation.

b) Generalised seizures

Absences (typical or atypical)

Generalised tonic clonic

Tonic

Clonic

Myoclonic

Atonic

Infantile spasms.

c) Unclassified aversive seizures are a form of simple partial seizure,


consisting of head turning and conjugate eye movements.
Rasmussen's encephalitis is a subacute inflammatory encephalitis, and is one
cause of epilepsia partialis continua.
Complex partial seizures often contain automatisms which may be
elementary (including lip smacking, chewing, swallowing or salivation), or
automatic behaviour (semi-purposive unco-ordinated or unplanned gestures
including picking and pulling at clothing).
Rolandic epilepsy is a benign partial epilepsy associated with centro-temporal
spikes. There is an excellent prognosis.
Further reading:
Lennox-Gastaut syndrome GP Notebook
2002 Dr Colin Melville

39-Maturity onset diabetes of the young (MODY) is due to which of the


following?

(Please select 1 option)

BRCA1 and BRCA2 (breast cancer) gene products


Glucokinase mutations

Correct

HOX (homeobox) gene family


Leptin mutations
Stargardt's disease mutations

MODY is a an autosomal dominantly inherited form of diabetes and is due to


glucokinase mutations.
Other causes of MODY include hepatocyte nuclear factor (HNF) gene
mutations.
Further reading:
Maturity-onset diabetes of the young; MODY NCBI National Center for
Biotechnology Information

40-The following statements regarding intravenous solutions are correct:


True / False

Normal saline contains 180 mmol/l of sodium

Correct

Ringer's lactate solutions is designed for extracellular fluid replacement


Sodium bicarbonate 8.4% is a hyperosmolar solution

Correct

Correct

0.18 saline in 4.0% dextrose is appropriate for the initial management of an infant
with signs of peripheral circulatory failure

Incorrect answer selected

Normal saline with added potassium is appropriate therapy to correct a nonrespiratory alkalosis

Incorrect answer selected

Normal saline contains 154 mmol/l of sodium.


Ringer's solution is also known as Hartmann's solution. It is a crystalloid and
contains 131 mmol/l of sodium and 111 mmol/l of chloride. It is ideally
balanced. In an extracellular loss such as diarrhoea or haemorrhage sodium
falls and therefore administration of a hyponatraemic solution may further
compound the problem. Ringer's is more appropriate.
Sodium bicarbonate 8.4% is a hyperosmolar solution with an osmolarity of
approximately 2000 mOsmol/l.

0.18% saline in 4% dextrose is also called dextrose saline. It is a crystalloid


containing 30 mmol/l of sodium and 30 mmol/l Cl. A child showing signs of
hypovolaemia or signs of a peripheral circulatory collapse needs to be
resuscitated with plasma expanders preferably. These include colloid fluids
such as blood or plasma. They exert an oncotic pressure and thus retain fluid
in the circulating volume. Crystalloid fluids containing iso or hypotonic
concentrations of sodium do not remain in the plasma volume following IV
administration. The volume of distribution of these fluids is extracellular and
thus only provides a short term expansion of the circulating volume.
Normal saline with additional potassium is used in the correction of metabolic
alkalosis.

41-A 39+5/40 gestation infant is born weighing 3.96kg by elective


caesarian section (previous section) under spinal anaesthesia. She is born in
good condition with Apgars of 9 at 1 minute and 10 at 5 minutes. She begins
grunting from birth, and after transfer to the neonatal unit requires 25%
Oxygen to maintain saturations of 94%.
On examination she is apyrexial, well and well perfused. Her respiratory
distress and grunting resolve over the next 12 hours, and she is out of
oxygen by the next morning.
What is the most likely diagnosis?
(Please select 1 option)

Congenital diaphragmatic hernia


Congenital pneumonia
Meconium aspiration
Surfactant-deficient lung disease
Transient tachypnoea of the newborn

Correct

The history is of CS delivery and transient respiratory distress with low O2


requirements, which resolves within the first 12-24 hours of birth. This is
characteristic of Transient Tachypnoea of the newborn, which is caused by retained
lung fluid. It often occurs in CS babies, in whom lung fluid has not been removed by
passage through the birth canal.

42-An 18 year-old female presents 12 weeks into an unplanned pregnancy.


She had been diagnosed with epilepsy 6 years ago which was well controlled
on Sodium Valproate and had been taking the combined oral contraceptive
pill for three years.
Which of the following is correct concerning this patient?
(Please select 1 option)

Lamotrigine should be substituted for Sodium Valproate


Sodium Valproate interaction with the oral contraceptive increased the risk of pregnancy.
She should be advised to have a termination of her pregnancy.
The dose of Sodium Valproate should be increased.
There is an increased risk of a neural tube defect in her foetus.

Correct

This patient has become pregnant on valproate. This therapy has controlled her
seizures and should not be changed now. However, there is an increased risk of
neural tube defects associated with valproate and this could be reduced by folate
therapy. Valproate is not an enzyme inducer and unlike other anticonvulsants would
not speed up metabolism of the OCP. Its entirely up to the individual if they wish to
pursue the pregnancy or not.

43-You are asked advice by a young professional couple, Mr and Mrs X. Mrs
X is 9 weeks pregnant. Mr X's brother and his partner had a child with cystic
fibrosis. As a result, Mr X was screened and found to carry the DF508
mutation for cystic fibrosis. Mrs X declines to be tested. What are the
chances of Mr and Mrs X's child having cystic fibrosis, given that the gene
frequency for this mutation in the general population is 1/20.
(Please select 1 option)

1/4
1/20
1/40
1/80

Correct

1/160

The chance of Mrs X being a carrier of the gene is 1/20. The chances of two carriers
of a recessive gene having a child that is homozygous for that disease (i.e. both
genes are transmitted to the child) is . Therefore, the chances of this couple having
a child with CF are x 1/20 = 1/80.

44-The thyroid hormone receptor is:


(Please select 1 option)

A gated ion channel


A cell surface receptor
A cytoplasmic protein
A G-protein coupled receptor
A nuclear receptor

Correct

The thyroid hormone receptor is a nuclear receptor. When it binds T3 it is able to


bind to the thyroid hormone response element (TRE) in the promoter region of
thyroid hormone responsive genes and initiates transcription.

45-A 35-year-old man presents with an inherited neurological disorder. His father developed
the disease in his 60s and his daughter was born 2 years ago with a severe form of the
condition. His mother, sister, wife and other child, a son, are unaffected. What is the genetic
basis underlying this condition?
(Please select 1 option)

Autosomal recessive inheritance


Mitochondrial disease
Polygenic inheritance
Trinucleotide repeat disease

Correct

X-linked inheritance

The inheritance from this man's father, to himself and then to his daughter shows
increasing disease severity and earlier onset of disease in subsequent generations.
This is genetic anticipation and is typical of trinucleotide repeat disease where there
is expansion of the repetitive sequence of three nucleotides with each generation.
Also the length of the expansion increases as cells divide through an individual's life.
Trinucleotide repeat diseases include Huntington's disease, myotonic dystrohpy,
fragile X syndrome, and Friedriech's ataxia.

46-An 18 year-old female is admitted with a depression of her conscious


level. Arterial blood gas analysis revealed:
pH

7.26 (7.36-7.44)

pO2

12.1 kPa (11.3-12.6)

pC02

3.9 kPa (4.7-6.0)

Standard bicarbonate 14.7 mmol/L (20-28)

Which one of the following would account for these results?


(Please select 1 option)

Analytical error
Metabolic acidosis

Correct

Persistent vomiting
Respiratory acidosis
Respiratory alkalosis

This patient has a metabolic acidosis with an effort at respiratory


compensation as reflected by elevated pO and reduced pCO . This could be
due to poisoning or a condition such as diabetic ketoacidosis. Vomiting would
cause a metabolic alkalosis.
2

47-A 3 week old girl on the neonatal unit is noted to have a fever and
decreased spontaneous movement. She was born at 26/40 gestation
weighing 1.02kg. After a stormy course she has returned from the neonatal
intensive care for convalescence. She is unimmunised. There is no FH/SH of
note.
fever of

37.6C

Respiratory rate 30/min


Heart rate

100/min

She has multiple venepuncture sites, and has redness over a site over the
dorsum of the right foot and over the shin, which is swollen. She cries when
this area is touched.lies still in her cot and she appears well perfused.
What is the most likely diagnosis?
(Please select 1 option)

Bacteraemia
Cellulitis

Incorrect answer selected

Osteomyelitis

This is the correct answer

Rickets of prematurity
Septic arthritis

The history is of low grade fever with pseudoparalysis of the right leg and tibial
inflammation. This suggests an osteomyelitis in the right tibia complicating a
previous infected drip site in the foot. USS, X-ray, bone scan and blood cultures
should be performed and IV antibiotics given to cover Staphylococcus aureus, Group
B streptococcus and gram negative organisms.

48-A 17-year-old male with learning difficulties is brought for review by his
worried parents after he described acute blurring of vision in his right eye.
Examination reveals ectopia lentis. What is the most likely diagnosis?
(Please select 1 option)

Ehlers-Danlos syndrome
Homocystinuria

Correct

Maple syrup urine disease


Marfan's syndrome
Metachromatic leukodystrophy

Ectopia lentis/subluxation of the lens is associated with

Ehlers-Danlos syndrome

Marfan's syndrome

Weill-Marchesani syndrome (short stature, skeletal


abnormalities and ectopia lentis)

Refsum's disease

but in this case homocystinuria is likely due to the associated mental


retardation.
Patients typically have fair skin with

coarse hair

osteoporosis

mental retardation (nearly 50%)

seizure disorder

Marfanoid habitus

increased thromboembolic risk.

Metachromatic leukodystrophy is a lysosomal storage disorder, characterised


by lipid (sulfatide) accumulation in the CNS, and associated with
psychomotor retardation in infants with optic atrophy and blindness.

49-Which of the following clinical manifestations suggests Guillain Barr


Syndrome?
(Please select 1 option)

Weakness beginning in the arms


Asymmetrical involvement of distal muscles
Bulbar involvement in about 50% of cases

Correct

Brisk tendon reflexes


Normal CSF protein

GB is a post-infectious polyneuropathy causing demyelination in mainly


motor but also sensory nerves. It usually follows a non-specific viral
infection. Campylobacter and mycoplasma are recognised causes. Weakness
begins in the legs and progressively ascends to involve the trunk, upper
limbs and finally the bulbar muscles (Landry's ascending paralysis).
Asymmetry is present in only 9% of patients, with symmetrical involvement
being typical. Usually there is painless progression over days or weeks, but in
cases of abrupt onset, there may be tenderness or muscle pain. Bulbar
involvement occurs in 50%, with a risk of aspiration and respiratory
insufficiency can be problematic. In the Miller Fisher Syndrome there is
external ophthalmoplegia, ataxia and areflexia. In 20% of cases there is
urinary incontinence of retention. Clinical symptoms usually improve within
2-3 weeks, though a chronic relapsing form is recognised. CSF protein is
elevated to more than twice the upper limit of normal, with normal glucose
and no pleocytosis. Bacterial cultures are negative and viral cultures rarely
isolate anything. The dissociation between a high CSF protein and a lack of
cellular response in a person with an acute or subacute polyneuropathy is
diagnostic of Guillain Barr Syndrome.

50-Which one of the following has its own self-replicating DNA?


(Please select 1 option)

Golgi body
Lysosomes
Mitochondria

Correct

Peroxisome
Rough endoplasmic reticulum

Abnormalities of mitochondrial deoxyribonucleic acid (DNA) are associated


with inherited conditions such as Leber's optic atrophy, mitochondrial
myopathy, lactic acidosis and stroke-like episodes (MELAS) syndrome, and
diabetes insipidus, diabetes mellitus, optic atrophy and deafness (DIDMOAD).
(Dr Vajira H. W. Dissanayake)

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