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一名患有迟发性X连锁脊椎骨骺发育不良的患者出现了以前未报道过的真皮基底膜和胶原纤维异常。

Previously unreported abnormalities of dermal basement membranes and collagen fibrils in a patient with X-linked spondyloepiphyseal dysplasia tarda.

作者信息

Borsey D Q, Hopwood D, Montgomery A J, Walsh D B, Jung R T

出版信息

Postgrad Med J. 1986 Oct;62(732):943-6. doi: 10.1136/pgmj.62.732.943.

DOI:10.1136/pgmj.62.732.943
PMID:3774727
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC2419045/
Abstract

Electron microscopic findings of the skin biopsy of a boy with spondyloepiphyseal dysplasia tarda showed several novel features. The basement membrane of the epidermis showed focal irregular thickening and reduplication. The collagen fibres showed foci of fibrous long spacing collagen and marked variation in fibre diameter. Taken together these abnormalities have not been reported in any other condition.

摘要

一名患有迟发性脊椎骨骺发育不良男孩的皮肤活检电子显微镜检查结果显示出几个新特征。表皮基底膜显示局灶性不规则增厚和重复。胶原纤维显示出纤维性长间距胶原灶以及纤维直径的显著变化。综合来看，这些异常在其他任何病症中均未被报道过。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a286/2419045/1e302768115e/postmedj00104-0041-b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a286/2419045/7b7579a61017/postmedj00104-0040-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a286/2419045/2ac113748806/postmedj00104-0040-b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a286/2419045/a24dc3857961/postmedj00104-0041-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a286/2419045/1e302768115e/postmedj00104-0041-b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a286/2419045/7b7579a61017/postmedj00104-0040-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a286/2419045/2ac113748806/postmedj00104-0040-b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a286/2419045/a24dc3857961/postmedj00104-0041-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a286/2419045/1e302768115e/postmedj00104-0041-b.jpg

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