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7 Cards in this Set

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Molecular Defect in Burton's hypogammaglobulinemia?
X-linked recessive defect in a Tyrosine Kinase gene (BtK gene)
Sx of Burton's Hypogammaglobulinemia?
Recurrent bacterial infections after 6 Mo; Low leveles of all calsses of IG;mB-cell cell maturation in bone marrow stops at pre-B stage, NML CMI
Tx of Burton's
Monthly gammaglobulin replacement and abx for infection
Sx of selective IgA deficiency?
Recurrent Sinopulmonary and GI infections
Tx for Selective IgA deficinecy (MC primary immunodeficinecy)
Abx, not immunoglubulins
Molecular defect causing Hyper-IgM syndrome?
Defect in CD40L (ligand)costimulatory molecule on CD4 T helper cells leading to inability to class switch since no stimulatory signal given to B cell.
Sx of Hyper-IgM syndrome?
High IgM titers, very low IgG, IgE, IgA
Children present with recurrent resp infections bacterial and PCP.
T-cell independent Ag (i.e.LPS) Not affected