RENAL TUBULAR DYSGENESIS ASSOCIATED WITH RENAL-VEIN THROMBOSIS

被引:3
|
作者
ORTMANN, M [1 ]
QUERFELD, U [1 ]
STOLLORZ, M [1 ]
SCHRODER, R [1 ]
机构
[1] UNIV COLOGNE,KINDERKLIN,COLOGNE,GERMANY
来源
PATHOLOGE | 1995年 / 16卷 / 02期
关键词
RENAL TUBULAR DYSGENESIS; AUTOSOMAL RECESSIVE DISORDER; OLIGOHYDRAMNIOS; POTTER SEQUENCE; FETAL RENAL VEIN THROMBOSIS;
D O I
10.1007/s002920050087
中图分类号
R36 [病理学];
学科分类号
100104 ;
摘要
Renal tubular dysgenesis is a rarely recognized condition characterized by oligohydramnios, Potter's sequence and congenital anuria leading to stillbirth or neonatal death from respiratory failure. It is thought to be inherited in an autosomal recessive manner. Definitive diagnosis is based on renal histology, revealing the lack of proximal tubule differentiation. Two additional cases of affected sibs in a family with parental consanguinity are reported. Lectin and immunohistochemical studies confirmed structural and functional immaturity of the proximal tubule. Further findings include bilateral renal vein thrombosis. The clinical and morphological parameters defining this disorder and the possible mechanisms of pathogenesis are discussed.
引用
收藏
页码:143 / 146
页数:4
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