Pathology EMQ template

 

YourName: Harsita Patel         

 

Theme: Primary Immune Deficiencies 1

 

OPTION LIST

 

A	IgA deficiency	I	X-linked SCID
B	Hyper IgM syndrome	J	Bare lymphocyte syndrome I
C	Kostmann syndrome	K	IFN gamma deficiency
D	Cyclic neutropenia	L	T-cell deficiency
E	Complement deficiency	M	C-1 inhibitor deficiency
F	Chronic Granulomatous disease	N	Di George syndrome
G	Antibody deficiency	O	IL12 receptor deficiency
H	Leukocyte adhesion deficiency	P	Bare lymphocyte syndrome II

 

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.    A lady comes in with her unwell 3 month old child who has been suffering from recurrent infections. On imaging he is found to have sclerosing cholangitis and further tests reveal the presence of T cells (mainly CD8+ population), B cells and IgM, however, IgG is absent.

2.    The prevalence of this condition is 1:600, where 2/3 of patients are asymptomatic, while a 1/3 have recurrent respiratory tract infections.

3.    A 32 year old man presents with local swelling due to leakage of fluid from blood vessels into connective tissue. One of the doctors tells the medical student that this is “Quincke’s edema”.

4.    A 2 year old boy is brought into A&E and diagnosed with PCP infection after suffering from recurrent bacterial infections and failure to thrive.

5.    A young child with congenital heart disease is has been having recurrent infectious. Investigations show the presence of B cells and IgM, but CD4, CD8 T-cells, and IgG are absent.

 

ANSWERS

1. J

2. A

3. M

4. B

5. N