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胸骨-肋骨-锁骨骨质增生症:一种迄今未被描述的病症(作者译)

[Sterno-costo-clavicular hyperostosis: a hitherto undescribed entity (author's transl)].

作者信息

Köhler H, Uehlinger E, Kutzner J, Weihrauch T R, Wilbert L, Schuster R

出版信息

Dtsch Med Wochenschr. 1975 Jul 18;100(29):1519-23. doi: 10.1055/s-0028-1106414.

Abstract

Persistent pulling pain in the sternum, clavicles and both first ribs, especially during cold and wet weather, necessitated medical investigations and treatment in 3 patients. Externally a cylindrical or spindle-shaped swelling of the clavicles was noticeable and two patients had signs of superior caval obstruction in addition. X-rays showed hyperostosis of the sternal and middle part of the clavicle, synostosis of the sternoclavicular joints with involvement of the two first ribs as well as a thickening and increase in breadth of the sternum. In one patient there were additional swellings of the first to fourth ribs bilaterally. During the period of observation there was no radiological progression of the bone disease. Apart from a consistently raised erythrocyte sedimentation rate blood chemistry was normal. The sterno-costo-clavicular hyperostosis led to bilateral occlusion of the subclavian veins with resulting upper venous congestion in two patients. Histology of the biopsy samples showed a characteristic hyperostotic spongiosclerosis in all three cases. The cause of this sterno-costo-clavicular hyperostosis is unknown. Clinical, radiological and histological findings indicate that it is a uniform disease which has not previously been described.

摘要

3例患者出现胸骨、锁骨及双侧第一肋骨持续性牵拉痛,尤其在寒冷潮湿天气时,因此需要进行医学检查和治疗。从外部可见锁骨呈圆柱形或纺锤形肿胀,另外两名患者还有上腔静脉阻塞的体征。X线显示胸骨及锁骨中部骨质增生,胸锁关节融合并累及双侧第一肋骨,同时胸骨增厚、增宽。1例患者双侧第一至第四肋骨额外出现肿胀。在观察期间,骨病无放射学进展。除红细胞沉降率持续升高外,血液生化指标正常。胸骨-肋骨-锁骨骨质增生导致两名患者双侧锁骨下静脉闭塞,进而引起上腔静脉淤血。活检样本的组织学检查显示,所有3例均有特征性的骨质增生性海绵状硬化。这种胸骨-肋骨-锁骨骨质增生的病因不明。临床、放射学和组织学检查结果表明,这是一种以前未被描述过的单一疾病。

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