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马罗-拉米综合征(黏多糖贮积症VI型)中Reilly小体(异染性颗粒)的超微结构。一项组织化学研究。

Ultrastructures of Reilly bodies (metachromatic granules) in the Maroteaux-Lamy syndrome (mucopolysaccharidosis VI). A histochemical study.

作者信息

Levy L A, Lewis J C, Sumner T E

出版信息

Am J Clin Pathol. 1980 Mar;73(3):416-22. doi: 10.1093/ajcp/73.3.416.

DOI:10.1093/ajcp/73.3.416
PMID:6767391
Abstract

The leukocytes from an 8-year-old white girl having features of the Maroteaux-Lamy syndrome (mucopolysaccharidosis VI) contained large granular inclusions (Reilly granules). Light and electron microscopy showed that these granules had characteristics of those seen in several other types of mucopolysaccharidoses, including Morquio's syndrome, which are of a different structure than those of other types of mucopolysaccharidosis. Similar granules were seen in the platelets, a finding which, so far as we know, has never been reported before. The granules contained an acid mucosubstance demonstrated by cytochemical studies.

摘要

一名8岁白人女孩患有马罗托-拉米综合征(黏多糖贮积症VI型),其白细胞含有大颗粒包涵体(赖利颗粒)。光镜和电镜检查显示,这些颗粒具有在其他几种黏多糖贮积症中所见颗粒的特征,包括莫尔基奥综合征,其结构与其他类型的黏多糖贮积症不同。在血小板中也发现了类似的颗粒,据我们所知,这一发现此前从未有过报道。细胞化学研究表明,这些颗粒含有酸性黏液物质。

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