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What is Adenosine deaminase (enzyme) deficiency is associated with: and explain in detail?
SCID- defect in common gamma chain of IL2 receptor along with IL-4,7 2nd M/C defect is -ADA deficiency total absence of T and B cell deficiency of PNP (purine nucleoside phosphorylase) X-linked agammaglobulinemia/ bruttons agamma globulinemia- defect in B tyrosine kinase gene pro B cell to pre B cell formation defect Transient hypogammaglobulinemia- delayed onset of normal IgG synthesis, self limiting. Chronic granulomatous disease- deficiency of NADPH oxidase. led to reccurent catalase positive infection eg.:-staph.