Rodríguez J I, Fraga J, Perera A
An Esp Pediatr. 1982 Mar;16(3):238-45.
The first report of dysplasia epiphysialis hemimelica in the spanish literature is presented. The case has peculiar clinical features as the early presentation (two months) and to involve lower and upper extremities. On X-ray examination exostosis and calcium deposits were shown on the most affected epiphysis. Microscopically an osteochondroma, anomalous vascular channels, non hitherto described, were detected, as well as multivacuolated cells into them although ultrastructural and histochemical studies were carried on the precise nature of these cells could not be established. The possible pathogenetic significance of these findings is discussed.
本文介绍了西班牙文献中关于半侧肢体骨骺发育异常的首例报告。该病例具有特殊的临床特征,如发病早(两个月)且累及上下肢。X线检查显示,最受影响的骨骺有外生骨疣和钙沉积。显微镜下发现了一个骨软骨瘤、异常血管通道(此前未描述过)以及其中的多泡状细胞,尽管进行了超微结构和组织化学研究,但仍无法确定这些细胞的确切性质。本文讨论了这些发现可能的致病意义。