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一名男孩出现手足异常、视网膜发育不良和难以解决的喂养困难,被诊断为 20q11.2-q12 新发缺失。

A de novo deletion of 20q11.2-q12 in a boy presenting with abnormal hands and feet, retinal dysplasia, and intractable feeding difficulty.

机构信息

Hiroshima Municipal Center for Child Health and Development, Hiroshima, Japan.

出版信息

Am J Med Genet A. 2011 Feb;155A(2):409-14. doi: 10.1002/ajmg.a.33818. Epub 2011 Jan 13.

Abstract

Proximal interstitial deletions involving 20q11-q12 are very rare. Only two cases have been reported. We describe another patient with 20q11.21-q12 deletion. We precisely mapped the 6.5-Mb deletion and successfully determined the deletion landmarks at the nucleotide level. Common clinical features among the three cases include developmental delay, intractable feeding difficulties with gastroesophageal reflux, and facial dysmorphism including triangular face, hypertelorism, and hypoplastic alae nasi, indicating that the 20q11.2-q12 deletion can be a clinically recognizable syndrome. This is also supported by the fact that the three deletions overlap significantly. In addition, unique features such as arthrogryposis/fetal akinesia (hypokinesia) deformation and retinal dysplasia are recognized in the patient reported herein.

摘要

涉及 20q11-q12 的近端染色质间缺失非常罕见。仅有两例报告。我们描述了另一位患有 20q11.21-q12 缺失的患者。我们精确地定位了 6.5Mb 的缺失,并成功地在核苷酸水平上确定了缺失的标志。三个病例的共同临床特征包括发育迟缓、难以控制的喂养困难伴胃食管反流,以及面部畸形,包括三角脸、眼距过宽和鼻侧发育不全,表明 20q11.2-q12 缺失可能是一种具有临床识别特征的综合征。这也得到了三个缺失显著重叠的事实的支持。此外,在本文报道的患者中还可以识别到关节挛缩症/胎儿运动不能(运动减少)畸形和视网膜发育不良等独特特征。

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