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哈伊杜-切尼综合征

Hajdu-Cheney syndrome.

作者信息

Iwaya T, Taniguchi K, Watanabe J, Iinuma K, Hamazaki Y, Yoshikawa S

出版信息

Arch Orthop Trauma Surg (1978). 1979;95(4):293-302. doi: 10.1007/BF00389701.

Abstract

Hadjdu-Cheney syndrome is a rare type of syndrome characterized by acro-osteolysis, dolichocephaly with multiple Wormian bones, absence of frontal sinuses and joint laxity. A case of this syndrome is presented. A histological study of the osteolytic lesion revealed destruction characterized by microfractures with a poor reparative process. It is postulated that an abnormality of osteoblast or osteoid function is the pathogenesis of this syndrome.

摘要

哈杰杜-切尼综合征是一种罕见的综合征,其特征为肢端骨质溶解、长头畸形伴多发缝间骨、额窦缺如及关节松弛。本文报告一例该综合征病例。对骨质溶解病变的组织学研究显示,其破坏特征为微骨折且修复过程不佳。据推测,成骨细胞或类骨质功能异常是该综合征的发病机制。

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