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7 Cards in this Set
- Front
- Back
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Molecular Defect in Burton's hypogammaglobulinemia?
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X-linked recessive defect in a Tyrosine Kinase gene (BtK gene)
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Sx of Burton's Hypogammaglobulinemia?
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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
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Tx of Burton's
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Monthly gammaglobulin replacement and abx for infection
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Sx of selective IgA deficiency?
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Recurrent Sinopulmonary and GI infections
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Tx for Selective IgA deficinecy (MC primary immunodeficinecy)
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Abx, not immunoglubulins
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Molecular defect causing Hyper-IgM syndrome?
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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.
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Sx of Hyper-IgM syndrome?
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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 |
