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一种与先天性手指屈曲挛缩相关的家族性关节病的病理特征。

Pathologic features of a familial arthropathy associated with congenital flexion contractures of fingers.

作者信息

Athreya B H, Schumacher H R

出版信息

Arthritis Rheum. 1978 May;21(4):429-37. doi: 10.1002/art.1780210405.

DOI:10.1002/art.1780210405
PMID:656159
Abstract

Three siblings with a recently recognized form of familial arthropathy were studied clinically and by light and electron microscopy of the synovium. On light microscopy, the synovium from all 3 children showed synovial hyperplasia, necrotic villi, deposition of eosinophilic and PAS-positive material, and large numbers of multinucleated giant cells. Electron microscopy of the synovium in one patient revealed giant cells with profuse rough and smooth endoplasmic reticulum. Some phagocytic cells contained degenerated collagen in vacuoles or invaginations. There was granular and fibrinlike material on the synovial surface, but no amyloid. This symmetrical arthropathy now reported in three families appears to have characteristic histological appearance of synovium that allows its identification as distinct from other known joint diseases.

摘要

对三名患有最近才被认识的一种家族性关节病的兄弟姐妹进行了临床研究,并对其滑膜进行了光镜和电镜检查。在光镜下,所有三名儿童的滑膜均显示滑膜增生、坏死绒毛、嗜酸性和PAS阳性物质沉积以及大量多核巨细胞。对一名患者的滑膜进行电镜检查发现巨细胞含有大量粗面和滑面内质网。一些吞噬细胞的液泡或内陷中含有变性的胶原蛋白。滑膜表面有颗粒状和纤维蛋白样物质,但无淀粉样蛋白。现在在三个家族中报告的这种对称性关节病似乎具有滑膜的特征性组织学表现,使其能够被识别为与其他已知关节疾病不同。

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Congenital contractural deformities of the fingers and arthropathy.手指先天性挛缩畸形与关节病
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