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一个患有IV型桡侧多指畸形的大家庭。

A large family with type IV radial polydactyly.

作者信息

Seyhan A, Akarsu N, Keskin F

机构信息

Department of Plastic and Reconstructive Surgery, Celal Bayar University, Manisa, Turkey.

出版信息

J Hand Surg Br. 1998 Aug;23(4):530-3. doi: 10.1016/s0266-7681(98)80139-3.

Abstract

This study examines one of the largest pedigrees with radial polydactyly type IV (uncomplicated polysyndactyly) comprising a total of 69 individuals, of whom 26 have been affected over six generations. Typical manifestations of the pedigree were bilateral radial and ulnar digital duplications, as well as syndactyly between the middle and ring fingers and the second and third toes. There was no craniofacial anomaly in any of the 17 cases examined physically. This observation suggests that radial polydactyly type IV and Greig craniofacial-synostosis syndrome with similar digital manifestations are clinically-distinct entities.

摘要

本研究调查了一个患有IV型桡侧多指(单纯性多指并指)的最大谱系之一,该谱系共有69人,其中26人在六代中受影响。该谱系的典型表现为双侧桡侧和尺侧手指重复,以及中指与环指之间、第二和第三趾之间并指。在接受体格检查的17例病例中,均无颅面异常。这一观察结果表明,具有相似手指表现的IV型桡侧多指和Greig颅面骨缝早闭综合征在临床上是不同的疾病。

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