Adenosine deaminase (enzyme) deficiency is associated with:
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.
Severe combined immunodeficiency (SCID)
X-linked agammaglobulinemia
Transient hypogammaglobulinemia of infancy
Chronic granulomatous disease
Immunology Pa 3 (Graft, Autoimmunity, Immunodeficiency Disorders)