先天发育性(骨)关节病──关节病的新概念  

Congenital Developmental (Osteo) Arthropathy: A New Conception of Arthropathy

作　　者：徐德永[1,2,3,4] 栾红梅[1,2,3,4] 冯卫华 孙锡和[1,2,3,4] 王波 张仙居[1,2,3,4] 

机构地区：[1]青岛医学院附属医院 [2]潍坊医学院附属医院 [3]青岛莱西市结核病防治所 [4]青岛即墨市人民医院

出　　处：《临床放射学杂志》1997年第4期233-236,共4页Journal of Clinical Radiology

摘　　要：目的：关节病的传统概念其病因是获得性的或由后天继发因素所致，本文提出先天发育性（骨）关节病的概念。材料与方法：本组通过对成骨不全（６例），Ｍａｒｆａｎ综合征（４例），粘多糖病（８例），多发骨骺发育异常（２２例），致密性骨发育不全（２例），干骺软骨发育异常（４例），石骨症（３例），遗传性多发外生骨疣（６例），内生软骨瘤病（４例），Ｍａｆｕｃｃｉ病（２例），系统性骨纤维发育异常（４例）和进行性骨化性肌炎（１例）等６６例骨发育异常患者的２５６份Ｘ线照片的回顾性复习，并与其临床表现作了对照分析。结果：发现这些患者在骨发育异常的同时，均伴有关节端的形态和关节的结构与功能异常，并且均在４０岁前（平均３０岁）出现关节退变表现。Objective: To introduce a new conception of arthropathy. A traditional conception of arthropathy is that the cause of joint disease are acquired or secondary.Materials and Methods:256 films of 66 patient with dysplasia of bone including: Osteogenesis imperfecta (n=6), Marfan syndrome (n=4), mucopolysaccharidoses (n=8), multiple epiphyseal dysplasia (n=22), pycnodysostosis (n=2), metaphyseal chondrodysplasia (n=4), osteopetrosis (n=3), hereditary multiple exostosis (n=6), enchondromatosis (n=4), maffucci disease (n=2), systemic fibrous dysplasia (n=4), and myositis ossificans progressive (n=1) were studied and compared with their clinical appearances retrospectively.Results:The results show that these patients diagnosed as osteodysplasia also have the abnormal shapes of articular faces, disordered structures and functions of joints. Moreover, their clinical manifestations were appeared before 40 year old (30 years).Conclusion: The arthropathy ought to be divided into two types: Congenital and acquired.

关 键 词：先天性 关节病 放射学诊断 

分 类 号：R684.04[医药卫生—骨科学] R816.8[医药卫生—外科学]