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1型原发性高草酸尿症的肝肾联合移植。骨组织病理学和草酸盐晶体含量。

Combined liver-kidney transplantation in primary hyperoxaluria type 1. Bone histopathology and oxalate body content.

作者信息

Toussaint C, Vienne A, De Pauw L, Gelin M, Janssen F, Hall M, Schurmans T, Pasteels J L

机构信息

Cliniques Universitaires de Bruxelles, Hôpital Erasme, Brussels, Belgium.

出版信息

Transplantation. 1995 Jun 27;59(12):1700-4. doi: 10.1097/00007890-199506270-00010.

Abstract

In three patients with end-stage renal failure due to primary hyperoxaluria type 1, successful combined liver-kidney transplantation enabled us to assess the insoluble oxalate pool, which was compared with the histopathological changes observed in iliac crest biopsy specimens. Good correlation was observed between the histopathological grade of bone oxalosis and the estimated oxalate content of the body. In the end-stage of oxalate bone disease, hyperparathyroidism does not play a significant role in bone resorption, which appears to be the consequence of the granulomatous reaction induced by oxalate deposition. Combined liver-kidney transplantation should be performed long before this stage. Early hepatorenal grafting in uremia secondary to primary hyperoxaluria type 1 would avoid the deleterious clinical consequences of systemic oxalosis and shorten the duration of postransplant hyperoxaluria, which may compromise the course of kidney graft.

摘要

在3例因1型原发性高草酸尿症导致终末期肾衰竭的患者中,成功的肝肾联合移植使我们能够评估不溶性草酸盐池,并将其与髂嵴活检标本中观察到的组织病理学变化进行比较。骨草酸沉着症的组织病理学分级与体内草酸盐估计含量之间存在良好的相关性。在草酸盐骨病的终末期,甲状旁腺功能亢进在骨吸收中不起重要作用,骨吸收似乎是草酸盐沉积诱导的肉芽肿反应的结果。肝肾联合移植应在此阶段之前很久进行。对于1型原发性高草酸尿症继发的尿毒症,早期肝肾移植可避免全身性草酸盐沉着症的有害临床后果,并缩短移植后高草酸尿症的持续时间,而后者可能会影响肾移植的进程。

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