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通过结膜活检的超微结构研究诊断溶酶体贮积病。

Diagnosis of lysosomal storage diseases by the ultrastructural study of conjunctival biopsies.

作者信息

Libert J

出版信息

Pathol Annu. 1980;15(Pt 1):37-66.

PMID:7443310
Abstract

This ultrastructural study of conjunctival biopsies of 150 patients affected with various lysosomal storage disease allows us to demonstrate particular lesions in all the infantile phenotypes investigated so far. These alterations appear before the first clinical signs, suggest the possibility of such a disorder, and are often specific enough to propose an accurate diagnosis either because the lysosomes have a typical appearance or because their topographic distribution among the different conjunctival cell types is characteristic. As the morphologic approach is an obligatory step in the recognition of lysosomal storage diseases, we suggest concurrent use of conjunctival biopsy for the early screening and diagnosis of these disorders, as it is a very simple, easy, harmless technique, whose results are generally superior to those obtained by other methods.

摘要

这项对150例患有各种溶酶体贮积病患者的结膜活检组织进行的超微结构研究,使我们能够在迄今为止所研究的所有婴儿型表型中证实存在特定病变。这些改变在首个临床症状出现之前就已出现,提示了此类疾病的可能性,并且往往具有足够的特异性,能够做出准确诊断,这要么是因为溶酶体具有典型外观,要么是因为它们在不同结膜细胞类型中的拓扑分布具有特征性。由于形态学方法是识别溶酶体贮积病必不可少的步骤,我们建议同时使用结膜活检来对这些疾病进行早期筛查和诊断,因为这是一种非常简单、容易且无害的技术,其结果通常优于其他方法所获得的结果。

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