Robles N R, Barquilla J F, Arrobas M, Campos de Orellana M C, González Ruiz C, Sánchez Casado E
Servicio de Nefrología, Hospital Regional Infanta Cristina, Badajoz.
An Med Interna. 1998 Jul;15(7):373-5.
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.
家族性膜增生性肾小球肾炎是一种罕见疾病，目前已报道8例。现描述2例新患者，为两兄弟，均为男性。活检结果为I型膜增生性肾小球肾炎。第三个兄弟死于终末期肾衰竭，但该患者未进行活检。对先前报道的该疾病病例进行了回顾，得出结论：所有已知病例均属于I型膜增生性肾小球肾炎且均为男性，提示为伴性隐性遗传。与散发性形式相比，低补体血症似乎不太常见。主要组织相容性抗原系统可能在发病机制中起作用，特别是HLA A2（以及具有交叉反应的如A28）和DQ7。
