IMMUNODEFICIENY DISORDERS

Question 0

Primary immunodeficiencies

Answer 0

B cell disorders, t cell disorders, combined b and t cells, phagocyte disorders, complement disorders

Question 1

Recurrent infections with pyogenic bacteria indicate a immunodeficiency in

Answer 1

B cells

Question 2

B cell disorders

Answer 2

X linked/ Bruton’s agammaglobinemia,selective IgA deficiency

Question 3

T cell disorders

Answer 3

DiGeorge Syndrome, chronic mucocutaneous candidiasis

Question 4

Combined B and T cell disorders

Answer 4

Severe combined immunodeficiency, wiskott aldrich syndrome, ataxia telangiectasia

Question 5

Phagocyte disorders

Answer 5

Chronic granulomatous disease, chediak higashi syndrome, leukocyte adhesion deficiency

Question 6

Complement disorders

Answer 6

Early- C2, C3; terminal- specific deficiency

Question 7

Very low levels all immunoglobulins; virtual absence of B cells due to tyrosine kinase mutation

Answer 7

Bruton’s aggamoglobinemia

Question 8

Mgmt for bruton’s

Answer 8

Pooled gamma globulin

Question 9

Selective IgA deficiency

Answer 9

Due to failure of isotype switching and result to recurrent bacterial sinus and lung infectiob

Question 10

Defect in B cell maturation to plasma cells

Answer 10

Common variable immunodeficiency

Question 11

Most common form of severe antibody deficiency affecting both children and adults

Answer 11

Common variable immunodeficiency

Question 12

Specific T cell deficiency for candida albicans

Answer 12

Chronic mucocutaneous candidiasis

Question 13

Defect jn IL2 receptors in T cells, ADA deficiency

Answer 13

SCID

Question 14

Inability to mount IgM response

Answer 14

Wiskott Aldrich syndrome

Question 15

Mutation in DNA repair enzymes

Answer 15

Ataxia telangiectasia

Question 16

Lack of NADPH oxidase activity

Answer 16

Chronic granulomatous disease

Question 17

Chronic granulomatous disease presents with recurrent infections of

Answer 17

Catalase positive bacteria, A. Fumigatis

Question 18

Failure of phagolysosomal fusion, faulty microtubules impair neutrophil chemotaxis

Answer 18

Chedak higashi syndrome

Question 19

Defective adhesion (LFA 1) proteins on the surface of phagocytes

Answer 19

Leukocyte adhesion deficiency

Question 20

Most common complement defect

Answer 20

C2 deficiency

Question 21

Recurrent infections due to stap aureus

Answer 21

C3 deficiency

Question 22

Specific deficiency in C5-C9

Answer 22

Terminal complement deficiency

Question 23

Bacteremia with N. Meningitidis or N. gonorrhea

Answer 23

Terminal complement def

Question 24

Complement responsible for opsonization

Answer 24

C3B

Question 25

Complementa that are anaphylatoxin

Answer 25

C3a, C4a, C5a

Question 26

Complement chemotactic to WBCs

Answer 26

C5a

Question 27

Complement wc is part of the membrane attack complex

Answer 27

C5b-C9