Pathology EMQ template.

Name: Carina Johnstone-Ayliffe

Candidate number:

Theme: Immune response to infection

OPTION LIST

A

X-linked SCID

I

Chronic granulomatous disease

B

DiGeorge syndrome

J

Kostmann’s syndrome

C

MHC class I deficiency

K

Leukocyte adhesion deficiency

D

MHC class II deficiency

L

Terminal pathway defect

E

Hyper IgM syndrome

M

Alternative pathway defect

F

Common variable ID

N

Classical pathway defect

G

Selective IgA deficiency

O

 

H

Bruton’s hypogammaglobulinaemia

P

 

For each scenario below, choose the most appropriate answer from the list above. Each option may be used once, more than once or not at all.

1. 14 year old girl presents with recurrent meningitis. C3 and C4 levels were normal. Both CH50 and AP50 were abnormal.

2. 4 year old boy presents with recurrent infections and abscess formation. Negative NBT test.

3. 5 year old girl presents with systemic vasculitis. She has a history of recurrent sinusitis and otitis media. Tests show a low CD8+ but normal CD4+ count.

4. 3 month old boy presents with failure to thrive, diarrhoea and recurrent infections. Tests showed low T cells and normal B cells.

5. 7 month old boy presents with recurrent respiratory tract infection with abscess formation, enteritis and malabsorption. Found to have no immunoglobulins or B lymphocytes.

ANSWERS

1. L

2. I

3. C

4. A

5. H