Bakkaloglu A, Söylemezoglu O, Tinaztepe K, Saatçi U, Söylemezoglu F
Pediatric Nephrology Department, Hacettepe University, Children's Hospital, Ankara, Turkey.
Nephrol Dial Transplant. 1995;10(1):21-4.
Four and two male sibs of two separate families who had biopsy-proven membranoproliferative glomerulonephritis (MPGN) are presented. In the first family four sibs of the first-degree consanguineous marriage showed the clinical picture of nephrotic syndrome without hypocomplementaemia at initial laboratory findings. In the second family two affected sibs showed nephrotic and nephritic syndromes on admission. Family investigations showed normal serum complement, immunoglobulins, T-cell subsets, urine analysis, and serum biochemistry. HLA typing in the two families revealed a common antigen HLA A2 in all affected sibs. Some other reports give suggestive evidence of MPGN in siblings but this is the first report that showed the occurrence of MPGN in four sibs. Our data strengthened the concept that genetic factors are involved in the development of MPGN but additional immunogenetic studies will shed light on the genetic aspects of the disease.
本文报告了两个独立家庭中分别有4名和2名男性同胞，他们经活检证实患有膜增生性肾小球肾炎（MPGN）。在第一个家庭中，一级近亲结婚的4名同胞表现出肾病综合征的临床症状，初始实验室检查时无补体降低。在第二个家庭中，2名患病同胞入院时表现为肾病综合征和肾炎综合征。家族调查显示血清补体、免疫球蛋白、T细胞亚群、尿液分析和血清生化指标均正常。两个家庭的HLA分型显示，所有患病同胞中都有一个共同抗原HLA A2。其他一些报告提供了兄弟姐妹中MPGN的提示性证据，但这是首次报告在4名同胞中发生MPGN。我们的数据强化了遗传因素参与MPGN发病的概念，但进一步的免疫遗传学研究将阐明该疾病的遗传方面。
