Immunodeficiencies

Question 1

what does IL4 tell you to switch too

Answer 1

IgG

Question 2

what does Il5 tell you to switch classes from IgM to?

Answer 2

IgA

Question 3

Explain x linked agammaglobulinemia

Answer 3

x linked cannot get mature b cells so no antibodies given loads of Immunogloblins to treat it

Question 4

what is common variable immunodeficiciency

Answer 4

you get mature b cells but not plasma cells so no antibodiesgamma globulins and anitobiotic therapy

Question 5

what is selective IgA deficiency immunodeficiciency

Answer 5

where you cannot produce IgA found in mucosal cells can be inherited or aquired through rubella infection asymptomatic but can be fatal to given plasma or breast milk so no gamma globulins.

Question 6

do people with selective IgA deficiency have cell surface IgA still?

Answer 6

yes they do but for some reason they cannot secrete the antibodies one in 600 caucasion s most common

Question 7

what is X linked hyper IgM syndrome?

Answer 7

caused by an inherited deficiency of CD40L on t cells, leads to no engaged B cells so no switching

Question 8

what causes hyper IgM type 2 syndrome

Answer 8

an inherited mutation in the gene cytidine deaminase that for some unknown reason prevents switching.

Question 9

what is digeorge syndrome or congenital thymic hypoplasia

Answer 9

missing part of all of the thymus at birth so no t cell maturation and stuggles with virus and fungi infections, could be caused by maternal alcoholism, chest xray to diagnos. Treat with aging by 5 the t cells level will increase.

Question 10

what is SCID

Answer 10

failure of B and T cells to develop from bone marrow stem cells. Usually die from infections within 1st year if not treated.

Question 11

what causes scid

Answer 11

25 percent of the time from deficiency of adenosine deaminase, x linked form is caused by a mutation in a chain common in many interluekins.

Question 12

what is the treatment for scid

Answer 12

bone marrow transplantation working on gene therapy to give the genes.

Question 13

what is chronic granulomatous disease

Answer 13

defenct in the NADPH oxidase means that you cannot produce superoxide anionssuring respitory burst in phagocytic cells. Treatment with bone marrow transplant, or some patients interferon gamma will work.

Question 14

what is myeloperoxidase deficiency

Answer 14

cannot make the HOCl from H2O2 not that big of a deal except with certain fungi infection candida yeast.

Question 15

what is chediak higashi syndrome

Answer 15

fusing of lysosomes in neutrophils making them less deadly, patients can have metallic silver hair, bleeding tendencies vision impairment, need a bone marrow transplant to treat it.

Question 16

leukocyte adhesion deficiency

Answer 16

defect in the LFA 1 so no adhesion of neutrophils in to the vasuclar wall so they build up in the bloodstream. Treatment with antibiotics, can ive granulocytes but will need a bone marrow transplant.

Question 17

what is wiskott aldrich syndrome

Answer 17

triad of symptoms. Immunodeficiency eczema and thrombocytopenia. Cytosolic protein b and t cells cant interact poor response to antigens. Treat with gamma globulin antibiotics and bone marrow cure immunodificiency.

Question 18

what is ataxia telangiectasia

Answer 18

recessive mutlisystem disease defect ATM needed for double stranded DNA breaks leads to tumors and lower t cells defective t helper cells low serum IgA IgE and IgG2, thymus remains in embryonic state treat with antibiotics and BMT will cure immune system defects.