HEREDITARY MULTIPLE EXOSTOSIS

HEREDITARY MULTIPLE EXOSTOSIS
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DOI:
10.1302/0301-620x.45b2.292
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发表时间:
1963-01-01
影响因子:
--
通讯作者:
SOLOMON, L
SOLOMON, L
中科院分区:
其他
文献类型:
--
作者:
SOLOMON, L

文献摘要

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对五十六例患者及其亲属进行了遗传性多发性外生骨疣的研究。在大多数情况下,以前的记录是可用的,疾病的进展可以追溯多年。特征性病变的描述和并发症中遇到的本系列的情况下指出。在大约三分之二的病例中,这种疾病是遗传的,并且总是在杂合子中产生可检测的损伤。软骨帽外生骨疣局限于内软骨骨骼,其发病率与所涉及部位的生长潜力密切相关。试图解释奇怪的分布外生骨疣,和可能的理论的发病机制的疾病进行了讨论。
Hereditary multiple exostosis has been studied in fifty-six patients and their relatives. In most cases previous records were available and the progress of the disease could be traced over many years. The characteristic lesions are described and the complications encountered in the present series of cases are noted. In approximately two-thirds of the cases the disease is inherited and invariably produces detectable lesions in the heterozygote. The Cartilage-capped exostoses are confined to the endochrondral skeleton where their incidence is closely related to the growth potential of the sites involved. An attempt has been made to explain the curious distribution of the exostoses, and the likely theories of the pathogenesis of the disease are discussed.