BIOL 2005 Lecture Notes - Antigen Processing, Hyper Igm Syndrome, Phos

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30 Jan 2013
Department
Course
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 adenoids (usually B-cell rich organs)!!
advantages of molecular genetic analysis:
pathophysiologic understanding
better delineation of typical vs. atypical patients
prenatal diagnosis/carrier detection
better therapy
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