FAMILIAL IGA NEPHROPATHY - EVIDENCE OF AN INHERITED MECHANISM OF DISEASE

FAMILIAL IGA NEPHROPATHY - EVIDENCE OF AN INHERITED MECHANISM OF DISEASE
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DOI:
10.1056/nejm198501243120403
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发表时间:
1985-01-01
影响因子:
158.5
通讯作者:
WYATT, RJ
WYATT, RJ
中科院分区:
医学1区
文献类型:
--
作者:
JULIAN, BA;QUIGGINS, PA;WYATT, RJ

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在来自美国肯塔基州中部和东部的伊加肾病患者中,对家族性肾小球肾炎进行了评价,发现了包含14例患者的潜在相关家系。另外17名家系成员患有临床肾小球肾炎,6名患有死亡证明上注明的“慢性肾炎”。6例伊加肾病患者有共同祖先。此外,6例患者的父母均来自其他伊加肾病病例的家庭。所有伊加肾病患者均未发现单一的HLA单倍型或抗原。这些家系的数据有力地支持了一些患者伊加肾病发病机制的遗传机制。
The evaluation of familial glomerulonephritis in patients with IgA nephropathy who were from central and eastern Kentucky [USA] resulted in the discovery of potentially related pedigrees containing 14 patients. An additional 17 members of the pedigrees had clinical glomerulonephritis, and 6 had "chronic nephritis" noted on their death certificates. Six patients with IgA nephropathy had a common ancestor. In addition, both parents of 6 patients with the disease came from families with other cases of IgA nephropathy. No single HLA haplotype or antigen was found in all the patients with IgA nephropathy. The data on these pedigrees strongly support an inherited mechanism in the pathogenesis of IgA nephropathy in some patients.