进行性遗传性肾脏病

• 作 者: 吴万龄
• 出 处: 国外医学参考资料(内科学分册)
• 发表日期: 1974年11期
• 关键词: 化验 常染色体 肾皮质 发病年龄 起病隐袭 程长 谱分析 髓质 尿液 囊肿

本文报告一种进行性遗传性肾脏病,其特征发病年龄常在40～50岁之间,起病隐袭,进展慢,病程长,尿化验正常,肾皮质髓质连接处可有囊肿,无失盐及严重贫血。家谱分析显示本病通过常染色体显性方式遗传(Autosomal dominant)。本文调查的同一世代12名亲族中,4人死于肾脏病,4人患有肾脏病。这4例尿液化验正常,仅一例

This article reports a progressive hereditary kidney disease, the characteristic age of onset is often between 40 to 50 years old, insidious onset, slow progress, long duration, normal urinalysis, renal cortex medulla junction may have cysts, no Loss of salt and severe anemia. Pedigree analysis shows that the disease is autosomal dominant. Of the 12 kin of the same generation investigated in this paper, 4 died of kidney disease and 4 had kidney disease. This 4 cases of urine test normal, only one case