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[肾小管发育不全]

[Renal tubular dysgenesis].

作者信息

Moldavsky M, Shahin A, Turani H

机构信息

Dept. of Pathology, Rebecca Sieff Government Hospital, Safed.

出版信息

Harefuah. 1995 May 1;128(9):542-3, 599.

PMID:7797153
Abstract

Renal tubular dysgenesis (RTD) is a very rare malformation, only 24 cases having been described. We present the case of twins born after 28 weeks gestation to a newly-arrived Ethiopian immigrant in whom oligohydramnios was diagnosed when she was admitted. 1 twin had developed polyhydramnios, died before birth and was born with hydrops fetalis. The other newborn suffered from pulmonary distress and anuria, and died 2 days after birth. At autopsy there were no apparent changes in the kidneys or urinary tract, but histological examination showed absence of proximal convoluted tubules. This was attributed to RTD and confirmed by histochemical examination.

摘要

肾小管发育不全(RTD)是一种非常罕见的畸形,仅报道过24例。我们报告了一名新到的埃塞俄比亚移民在妊娠28周后分娩的双胞胎病例,该孕妇入院时被诊断为羊水过少。其中1个胎儿出现羊水过多,出生前死亡,出生时患有胎儿水肿。另一个新生儿患有肺窘迫和无尿,出生后2天死亡。尸检时,肾脏和尿路未见明显变化,但组织学检查显示近端曲管缺如。这被归因于肾小管发育不全,并经组织化学检查证实。

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