Immunodeficiency Syndromes

Question 1

What is the defect in Chediak Higashi syndrome?

Answer 1

Defective fusion of Phagosomes and lysosomes, resulting in defective phagocytosis function and susceptibility to infection

Question 2

What is the defect in CGDs

Answer 2

Defects in microbicidal activity(phagocytic oxidase)….. The genes of phagolysosome enzyme system involves with oxidative burst are defective

Question 3

In X linked SCID, there is mutation in?

Answer 3

Gamma chain subunit of the cytokine receptors that transduce signals in IL2,4,7,9,11,15,21

Question 4

Interleukins…… and …….. are responsible for proliferation of Lymphoid progenitors and NK cells respectively

Answer 4

7 and 15

Question 5

Autosomal recessive SCID is the most common cause of?

Answer 5

Deficiency of Adenosine Deaminase, which leads to the accumulation of deoxyadenosine and derivatives which is toxic to dividing cells

Question 6

Manifestations of SCID includes?

Answer 6

Recurrent infections b4 the age of 6months by a wide range of pathogens

Question 7

An example of a Primary Immunodeficiency syndrome characterized by reduced T cells and reduced B cells are called……… and ……… respectively

Answer 7

X- linked SCID——reduced T cells, but B cell number may be normal

X- linked Agammaglobinemia of Bruton———-Absent or markedly reduced B cells, but T cells are normal

Question 8

A T cell deficiency due to non development of 3rd and 4th pharyngeal pouches is?

Answer 8

Di George syndrome

Question 9

Why does Tetany occur due to absent Parathyroid gland in Di George syndrome

Answer 9

Hypocalcemia dues to absence of PTH.
Hypocalcemia is the major cause of tetany

Question 10

An immunodeficiency in which B cells are able to proliferate in response to antigen but cannot produce immunoglobulins (plasma cells) is?

Answer 10

Combined Variable immunodeficiency

Question 11

The most common B cell immunodeficiency disorder is?

Answer 11

Isolated IgA deficiency

Question 12

A disorder characterized by inability of a patient to make antibodies to polysaccharide antigens is called?

Answer 12

Wiskott Aldrich Syndrome

Question 13

The risk of seroconversion after a needle stick accident in an HIV situation is?

Answer 13

About 0.3%

Question 14

The risk of seroconversion after accidental exposure to HBV is?

Answer 14

30%

Question 15

The most abundant viral antigen used in the diagnosis of HIV infection is?

Answer 15

P24 antigen