immunodeficiency II

Question 1

chromosome 22q11.2 deletion syndrome

Answer 1

di Geroge syndrome

Question 2

parathyroid hypoplasia

Answer 2

hypocalcemia

Question 3

cardiac anomalies, hypoplastic/absent thymus, hypocalcemia, facial abnormalities

Answer 3

Di George

Question 4

CATCH 22

Answer 4

C-cardiac abnormality 
A- abnormal facies
T- Thymic aplasia
C- Cleft palate
H- Hypocalcemia

Question 5

recurrent infections with intracellular bacteria, fungi, large viruses and also pyogenic organims due to lack of T cell help for the B cells

Answer 5

Di George

Question 6

Patients with Di George syndrome will have:

A)	Decreased numbers of surface IgM and IgD positive B-cells
B)	Normal serum IgE levels
C)	Normal serum anti-influenza IgG levels after immunization with the influenza vaccine
D)	Normal ability to produce acute phase proteins such as CRP
E)	Normal IL-2 production

Answer 6

D) Normal ability to produce acute phase proteins such as CRP

Question 7

both humoral and cell mediated immunity are defective and susceptible to all infections agents

Answer 7

SCID

Question 8

The comonest form of SCID is a mutation of the common-chain of the

Answer 8

IL-2 receptor

Question 9

Tx. for SCID

Answer 9

stem cell transplantation

Question 10

What do we need to worry by a stem cell transplantation

Answer 10

GVH disease

Question 11

ADA deficiency

Answer 11

SCID for adenosine deaminase deficiency

Question 12

SCID: _____ lymphocyte syndrome

Answer 12

Bare

Question 13

mutations of protein kinase (JAK3 or ZAP 70) or mutations in RAG1 and RAG2

Answer 13

abnormal signal syndrome–> SCID

Question 14

mutation of the CD3 molecule

Answer 14

SCID

Question 15

immunodeficiency, thrombocytopenia (low platelet count), Eczema

Answer 15

wiskott aldrige syndrome

Question 16

Ataxia, telangiectasia, immunodeficiency

Answer 16

Ataxia telangiectasia

Question 17

purpura

Answer 17

Wiscott-Aldrige

Question 18

Tx. of Wiscott-aldrige

Answer 18

stem cell transplant

Question 19

lymphoma, myeloma, Burns

Answer 19

defective humoral immunity

Question 20

patients taking immunosuppressive drugs, malnutrition, viral infections especially HIV, ageing

Answer 20

defective cell mediated immunity

Question 21

HIV infect the

Answer 21

CD4 cells

Question 22

Viral _________ binds to the CD4 molecule

Answer 22

gp 120

Question 23

Gp41 binds to chemokine receptor

Answer 23

CCR5

Question 24

can you detect HIV antigen or anitbody in the blood?

Answer 24

Yes

Question 25

CD4:CD8 in HIV

Answer 25

it is reversed, usually it is 2:1 but in HIV it is 1:2

Question 26

what do you need for a bone marrow match

Answer 26

both MHC I and MHC II

Question 27

how can you measure the progress the disease of HIV

Answer 27

HIV RNA

Question 28

associated with significant and continuous immune deficiency

Answer 28

ageing

Question 29

as we age we increase the number and function of many cell types including neutrophils, APC, NK and T-cells

Answer 29

FALSE; it decreases

Question 30

in chronic mucocutaneous candidiasis there is a lack of _______ production or autoantibody to _______

Answer 30

IL-17

Question 31

severe warts

Answer 31

specific immunodeficiency