Pathology EMQ template

 

YourName: Craig Nightingale    

YourIdentifier:

Theme: Primary Immune Deficiencies 1

 

OPTION LIST

 

A

Chronic granulomatous disease

I

Severe combined immunodeficiency

B

MBL deficiency

J

C3 deficiency

C

Leukocyte adhesion deficiency

K

Classical pathway deficiency

D

Reticular dysgenesis

L

IFN gamma deficiency

E

DiGeorge syndrome

M

Kostmann syndrome

F

Bare lymphocyte syndrome

N

Alternative pathway deficiency

G

Bruton’s x-linked hypogammaglobulinaemia

O

IL 12 receptor deficiency

H

Selective IgA deficiency

P

Hyper IgM syndrome

 

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. An angry youth presents to hospitals with new trainers and a plasma TV in tow, he is found to have high levels of inflammation with lymphadenopathy and hepatosplenomegaly. A nitroblue tetrazolium test is positive.

 

 

2. A young lady presents to her GP following a gruelling shift at OshKosh B’Gosh Babies and Kids, complaining of recurrent infections. On investigation, she is found to have no neutrophils, although leukocyte adhesion markers are normal. She reports never developing any pus with her infections.

 

 

3.  A strange man is admitted to A&E, he has doughnut icing around his groin and a BBQ tongs branding on his buttocks, he is slightly confused, but reports a history of persistent bacterial infections, although he doesn’t know the cause. His blood work returns and shows a normal inflammatory response, except that no membrane attack complexes are found.

 

 

4. A student presents to his GP with a history of regular infections since childhood. He has a high forehead with low set folded ears and a cleft palate, you notice he also has an abnormally small mouth and jaw. He complains that these infections are interfering with his “fetball” commitments. Blood tests show a reduced number of T cells in his circulation with low IgA and IgG, however B cells are normal.

 

 

5. Two parents bring their 8th child, a 6 month old baby, to the paediatrics unit as he is having chronic diarrhoea, ear infections and oral candidiasis. They constantly complain and are worried that this will affect their child benefits. The father is particularly worried as he remembers a family history of similar events and early childhood mortality. The child is found to have complete T and B lymphocytopenia with reduced IgM, IgG and IgA.

 

 

ANSWERS

1. A

2. M

3. J

4. E

5. I