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慢性肾衰竭;劳伦斯-穆恩-比德尔综合征的一个重要特征。

Chronic renal failure; an important feature of the Laurence-Moon-Biedl syndrome.

作者信息

Williams B, Jenkins D, Walls J

机构信息

Department of Nephrology, Leicester General Hospital, UK.

出版信息

Postgrad Med J. 1988 Jun;64(752):462-4. doi: 10.1136/pgmj.64.752.462.

Abstract

Two cases of end stage renal failure occurring in association with the Laurence-Moon-Biedl syndrome are reported. Abnormalities in renal function and morphology are increasingly recognized in these patients in whom uraemia is an important cause of morbidity and early mortality. The presence of renal impairment, occurring as frequently as any of the pentad of features that characterize the syndrome, has important implications for the prognosis and long term management of these patients.

摘要

报告了两例与劳-穆-比综合征相关的终末期肾衰竭病例。这些患者的肾功能和形态异常越来越受到重视,其中尿毒症是发病和早期死亡的重要原因。肾功能损害的存在与该综合征特征性五联征中的任何一项一样常见,对这些患者的预后和长期管理具有重要意义。

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本文引用的文献

3
Renal abnormalities in the Bardet-Biedl syndrome.
Int J Pediatr Nephrol. 1982 Sep;3(3):199-203.
7
Pathology of Laurence-Moon-Bardet-Biedl syndrome.
J Pathol Bacteriol. 1967 Jan;93(1):65-79. doi: 10.1002/path.1700930106.
8
Renal involvement in the Laurence-Moon-Biedl syndrome. Functional and radiological studies.
Acta Paediatr Scand. 1986 Mar;75(2):240-4. doi: 10.1111/j.1651-2227.1986.tb10192.x.
10
The renal lesion of the Laurence-Moon-Biedl syndrome.
J Pediatr. 1975 Aug;87(2):206-9. doi: 10.1016/s0022-3476(75)80580-4.

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