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宽拇指-拇趾(鲁宾斯坦-泰比)综合征,1957 - 1988年

Broad thumb-hallux (Rubinstein-Taybi) syndrome 1957-1988.

作者信息

Rubinstein J H

机构信息

University Affiliated Cincinnati Center for Developmental Disorders, Ohio 45229.

出版信息

Am J Med Genet Suppl. 1990;6:3-16. doi: 10.1002/ajmg.1320370603.

Abstract

This presentation records the early history of the description of the broad thumb-hallux syndrome and attempts to update the current state of knowledge about this syndrome. Information was collected and reviewed on 571 individuals from the world literature, from communications with colleagues and families of affected individuals, and from personal observation. The diagnosis was established in most cases by confirming the concurrence of the constellation of major diagnostic criteria, including broad short terminal phalanges of the thumbs and halluces, with or without angulation deformity; characteristic facial appearance with beaked or straight nose, antimongoloid slant of palpebral fissures, apparent or clinical hypertelorism and grimacing smile; stature and head circumference (OFC) below 50th centile; mental, motor, social, and language retardation; stiff awkward gait; and incomplete or delayed descent of testes in males. Information on associated clinical factors, familial occurrence, and cytogenetic findings is presented.

摘要

本报告记录了宽拇指-拇趾综合征描述的早期历史,并试图更新有关该综合征的当前知识状态。通过查阅世界文献、与受影响个体的同事和家属交流以及个人观察,收集并审查了571例个体的信息。在大多数病例中,通过确认主要诊断标准的组合来确立诊断,这些标准包括拇指和拇趾的宽短末节指骨,伴有或不伴有成角畸形;具有鹰嘴状或挺直鼻子、睑裂反蒙古样倾斜、明显或临床性眼距增宽和怪相微笑的特征性面容;身高和头围(OFC)低于第50百分位数;智力、运动、社交和语言发育迟缓;僵硬笨拙的步态;以及男性睾丸下降不全或延迟。还介绍了相关临床因素、家族发病情况和细胞遗传学发现的信息。

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