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BIOL 2005 (40)
Lecture

Immunodeficiency

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Department
Biology
Course
BIOL 2005
Professor
Elizabeth Nisbet
Semester
Fall

Description
IMMUNODEFICIENCY 150 known diseases 2 mechanisms of hypogammaglobulinemia  2 clinical manifestations 1) poor maturation of B cells: X-Linked Agammaglobulinemia prototypic antibody deficiency disease antibody protective functions: opsonization of bacteria virus neutralization (prevent binding) toxin neutralization activation of complement mouse model: Xid (x-linked immunodeficiency) **no B cells  no plasma cells  no antibodies** x-linked; heterozygous females are carriers and NORMAL FEATURES: Onset = 6 mos. (after passive immunity is gone) Infections = pneumococcus, influenza, staph (heavily encapsulated bacteria that can evade phagocytosis) enterovirus infection (ECHO, Coxsackie, Polio) fatal if untreated because virus is NEVER CLEARED Clinical course – recurrent combinations of pulmonary, ENT, GI infection chronic lung disease dermatomyositis (skin/musc inflamm.) due to viral inf. death largely due to new mutation (a fatal disease if untreated) afollicular lymph nodes, poor cortico-medullary differences no tonsils or aden
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