Abstract

Familial membranoproliferative glomerulonephritis is a rare disease of which eight cases has been reported. Two new patients are described now, two brothers, both of then males. Type I membrano-proliferative glomerulonephritis was the finding of biopsy. A third male brother died due to end stage renal failure, but biopsy was not performed in this patient. Previously reported cases of the disease are reviewed and it is concluded that all known cases belong to type I membranoproliferative glomerulonephritis and are males, suggesting a sex-linked recessive hereditary transmission. Hypocomplementemia seems to be less frequent than in the sporadic form. Major histocompatibility antigen system may have a role in the pathogenesis, specially, HLA A2 (and those with cross-reaction like A28) and DQ7.

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