Immunodeficiency and Infection

Question 1

What is severe combined immunodeficiency? (SCID)

Answer 1

Genetic defect leading to a lack of functional T and B cells in blood

Question 2

What is DiGeorge syndrome?

Answer 2

A developmental defect of the thymus, sx-hypoparathyroidism and congenital heart anomalies, tx-thymic transplantation and passive antibody treatment

Question 3

What is leukocyte adhesion deficiency?

Answer 3

Defect in phagocytic cells, susceptible to fungal and viral infections, T-B defect

Question 4

What is chronic granulomatous disease?

Answer 4

X-linked or autosomal recessive, neutrophil defect

Question 5

What is complement deficiency?

Answer 5

Defect in complement pathway, associated with inc susceptibility to bacterial infections

Question 6

How do we approach patients with PID?

Answer 6

Hx, examination and investigations like diagnostic tests

Question 7

How do we treat SCID?

Answer 7

Isolation, no live vaccine, blood product requirements, immunoglobulin therapy, enzyme replacement, definitive tx-haematopoietic stem cell transplant

Question 8

Whats is AIDs?

Answer 8

The loss of the immune function that results from exposure to external agents