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透析相关性淀粉样变的组织分布。

Tissue distribution of dialysis amyloidosis.

作者信息

Noël L H, Zingraff J, Bardin T, Atienza C, Kuntz D, Drüeke T

出版信息

Clin Nephrol. 1987 Apr;27(4):175-8.

PMID:3555909
Abstract

Twenty-three uremic patients on intermittent hemodialysis for eight to eighteen years provided the material for the present pathological study. In all of them, there was evidence for dialysis related amyloidosis based on previous clinical or histological findings or both. The material examined consisted of nine skin biopsies, five abdominal fat aspirates, eight trans-iliac bone biopsies and numerous post-mortem specimens of various visceral organs from eight cases. None of the skin biopsies or fat aspirates showed amyloid deposits. In only one bone biopsy could a small Congo red positive area be recognized that showed characteristic birefringence under polarizing light. Autopsy material findings were negative except for one case: this patient had been dialyzed for 18 years. Very minute amyloid deposits with a positive immunofluorescence staining for beta 2-microglobulin (beta 2-M) were found in the walls of small vessels from her lung, heart, liver and intestine. Thus, in chronic hemodialysis patients the accumulation of beta 2-M amyloid fibrils in tissues other than joints and juxta-articular structures appears to have a low incidence, to occur lately and to be of limited size. Although extra-articular amyloid deposits may progressively occur and extend with increasing survival time on dialysis, tiny deposits such as those observed in only two of our patients will hardly lead to serious complications.

摘要

23名接受间歇性血液透析8至18年的尿毒症患者为本次病理研究提供了材料。基于先前的临床或组织学发现或两者,所有患者均有透析相关性淀粉样变的证据。所检查的材料包括9份皮肤活检标本、5份腹部脂肪抽吸物、8份经髂骨活检标本以及来自8例患者的多个各种内脏器官的尸检标本。皮肤活检标本或脂肪抽吸物均未显示淀粉样沉积物。仅在一份骨活检标本中可识别出一个小的刚果红阳性区域,该区域在偏振光下显示出特征性双折射。尸检材料的结果均为阴性,仅有一例除外:该患者已接受透析18年。在其肺、心脏、肝脏和肠道的小血管壁中发现了非常微小的淀粉样沉积物,其β2-微球蛋白(β2-M)免疫荧光染色呈阳性。因此,在慢性血液透析患者中,β2-M淀粉样纤维在关节和关节周围结构以外的组织中的积累似乎发生率较低、出现较晚且范围有限。尽管关节外淀粉样沉积物可能会随着透析存活时间的延长而逐渐出现并扩展,但像我们仅在两名患者中观察到的微小沉积物几乎不会导致严重并发症。

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