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遗传性多发性骨软骨瘤的影像学表现及骨软骨瘤发病机制的新理论

Radiographic findings in hereditary multiple exostoses and a new theory of the pathogenesis of exostoses.

作者信息

Pazzaglia U E, Pedrotti L, Beluffi G, Monafò V, Savasta S

机构信息

Clinica Ortopedica, Università di Pavia, I.R.C.C.S. Policlinico San Matteo, Italy.

出版信息

Pediatr Radiol. 1990;20(8):594-7. doi: 10.1007/BF02129063.

Abstract

Analysis of 330 exostoses in 18 patients affected by hereditary multiple exostoses disease suggested a new classification of exostoses as eccentric or full-thickness. Radiographically arrest of metaphyseal remodeling with failure of coning and persistence of the primary metaphyseal trabeculae was evident in full-thickness exostoses. Similar bone lesions can be obtained experimentally with inhibitors of bone turn-over. A localized, peripheral defect in remodeling over a limited time can give a satisfactory explanation also for the origin of eccentric exostoses. The thesis that this is the basic mechanism of exostosis formation is presented.

摘要

对18例遗传性多发性骨软骨瘤病患者的330处骨软骨瘤进行分析,提示骨软骨瘤可分为偏心性或全层性的新分类。影像学上,全层骨软骨瘤可见干骺端重塑停滞,呈锥形失败,初级干骺端小梁持续存在。使用骨转换抑制剂进行实验可获得类似的骨病变。有限时间内局部、周边重塑缺陷也能对偏心性骨软骨瘤的起源给出满意解释。本文提出这是骨软骨瘤形成的基本机制这一论点。

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