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[儿童先天性上肢部分巨大症]

[Congenital partial gigantism of the upper extremities in children].

作者信息

Pavlova M N, Beliaeva A A, Gusev B V

出版信息

Arkh Patol. 1986;48(8):63-8.

PMID:3778237
Abstract

Angio-and phlebography, as well as pathomorphological studies were made on epiphyseal cartilaginous plate, diaphyseal bone tissue and skin of patients with partial gigantism of upper extremities. Significant developmental anomalies were found in the formation of palmar arches and digital arteries, as well as hypoplasia and aplasia of deep brachial and antebrachial veins. Light and electron microscopy revealed epiphyseal plate dystrophy and dysplasia, tubular bone osteoporosis, subcutaneous fat hyperplasia. The data obtained suggest a pathogenetic relationship between the vascular formation and the development of some malformations and growth abnormalities of upper extremities.

摘要

对上肢部分巨人症患者的骨骺软骨板、骨干骨组织和皮肤进行了血管造影和静脉造影以及病理形态学研究。发现掌弓和指动脉形成存在明显发育异常,以及肱深静脉和前臂静脉发育不全和缺如。光镜和电镜检查显示骨骺板营养不良和发育异常、管状骨骨质疏松、皮下脂肪增生。所获数据提示上肢某些畸形和生长异常的发生与血管形成之间存在发病机制上的关联。

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