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血色素沉着症的关节病

The arthropathy of hemochromatosis.

作者信息

Hirsch J H, Killien F C, Troupin R H

出版信息

Radiology. 1976 Mar;118(3):591-6. doi: 10.1148/118.3.591.

Abstract

Five cases of hemochromatosis arthropathy are presented and the distinctive radiological features of the disease are described. Although the condition is typically degenerative, showing subchondral cyst formation, sclerosis, and thinning of cartilage, its distribution is characteristic. Selective degenerative changes of the second and third metacarpophalangeal joints are striking, particularly in the hands, while abnormalities in the intercarpal joints are variable and the interphalangeal joints are spared. Chondrocalcinosis involving both fibrous and hyaline cartilage is frequently seen as well, particularly in the large joints. The calcification is due to deposition of calcium pyrophosphate crystals, perhaps resulting from iron inhibition of pyrophosphatase.

摘要

本文报告了5例血色素沉着症性关节病,并描述了该病独特的放射学特征。尽管该病通常为退行性病变,表现为软骨下囊肿形成、硬化和软骨变薄,但其分布具有特征性。第二和第三掌指关节的选择性退行性改变显著,尤其是在手部,而腕骨间关节的异常情况不一,指间关节则未受累。同时,纤维软骨和透明软骨的软骨钙质沉着也很常见,尤其是在大关节。这种钙化是由于焦磷酸钙晶体沉积所致,可能是铁抑制焦磷酸酶的结果。

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