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以色列社区中四种类型的生长激素相关侏儒症的发生情况。

Occurrence of four types of growth hormone-related dwarfism in Israeli communities.

作者信息

Adam A, Josefsberg Z, Pertzelan A, Zadik Z, Chemke J M, Laron Z

出版信息

Eur J Pediatr. 1981 Sep;137(1):35-9. doi: 10.1007/BF00441167.

Abstract

Data are presented on 121 dwarfed patients belonging to 98 Israeli families with 4 types of dwarfism related to deficiency or in activity of human growth hormone (hGH): isolated growth hormone deficiency (IGHD), partial hGH deficiency (pIGHD), multiple pituitary hormone deficiencies (MPHD) and Laron-type dwarfism (LTD). These series are believed to comprise most of the dwarfs of these types in Israel; however, their distribution among the various ethnic communities varies greatly: LTD (15 families) is confined to a few communities of Oriental Jews; only 3 of 30 families with IGHD, but 7 of 10 families with pIGHD, are Ashkenazi, whereas the clinic distribution of 42 families with MPHD corresponds roughly to that of the general population. These data seem to reflect the role of genetic factors in the etiology of the various types of dwarfism: the stronger the genetic component, the greater is its deviation from random occurrence among the various communities.

摘要

本文呈现了121例侏儒症患者的数据,这些患者来自98个以色列家庭,患有4种与人类生长激素(hGH)缺乏或活性异常相关的侏儒症:孤立性生长激素缺乏症(IGHD)、部分hGH缺乏症(pIGHD)、多种垂体激素缺乏症(MPHD)和拉伦型侏儒症(LTD)。据信,这些病例系列涵盖了以色列大多数此类侏儒症患者;然而,它们在不同种族群体中的分布差异很大:LTD(15个家庭)仅限于少数东方犹太人社区;30个IGHD家庭中只有3个是德系犹太人,但10个pIGHD家庭中有7个是德系犹太人,而42个MPHD家庭的临床分布大致与普通人群相符。这些数据似乎反映了遗传因素在各种类型侏儒症病因中的作用:遗传因素越强,其在不同群体中的分布偏离随机分布的程度就越大。

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