Genetic Defects of the Complement Pathways: Relationship to HLA and Disease
There are known and theoretical defects in the immune response system which might make an individual susceptible to the development of nephritis. Certainly the various deficiencies of immunoglobulins, B and T lymphocytes are well known, and are clinically characterized by recurrent infections. They are not known to be associated with renal diseases, other than perhaps urinary tract infections. Increasing interest has focused lately on inherited, isolated deficiencies of components of the complement system. While first recognized in otherwise normal individuals (reviewed in Jersild et al., 1976; Carpenter et al., 1977; Schur, 1977, 1978; Agnello, 1978), there have recently been numerous reports of their association with disease.
KeywordsMajor Histocompatibility Complex Complement Pathway Membranoproliferative Glomerulonephritis Hereditary Angioedema Complement Deficiency
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