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一名伊曼纽尔综合征患者的临床与分子分析

A clinical and molecular analysis of a patient with Emanuel syndrome.

作者信息

Luo Jin-Wen, Yang Huan, Tan Zhi-Ping, Tu Ming, Luo Hong, Yang Yi-Feng, Xie Li

机构信息

Department of Cardio‑Thoracic Surgery, Hunan Children's Hospital, Changsha, Hunan 410007, P.R. China.

Department of Respiratory Medicine, Hunan Provincial People's Hospital, Changsha, Hunan 410001, P.R. China.

出版信息

Mol Med Rep. 2017 Mar;15(3):1348-1352. doi: 10.3892/mmr.2017.6107. Epub 2017 Jan 5.

Abstract

Emanuel syndrome (ES) is the most frequent type of recurrent non‑Robertsonian translocation that is characterized by numerous anomalies. Over 100 patients with ES have been described in the literature. The phenotype of this syndrome varies but often consists of facial dysmorphism, microcephaly, severe intellectual disability, developmental retardation, congenital heart disease and genital anomalies. The present study describes a 2‑year‑old boy with multiple malformations, including facial dysmorphism, severe intellectual disability, growth retardation, congenital heart disease, cleft lip and palate, genital malformation (micropenis), amblyopia, thymic dysplasia and hearing impairment. The karyotype of the patient was 47,XY,+del(22)(q13), and the maternal karyotype was 46,XX,t(11;22)(q25;q13),9qh‑,15p+. Single‑nucleotide polymorphism‑array analysis of the proband indicated a partial duplication of chromosomes 22 and 11 at 22q11.1‑q11.21 and 11q23.3‑q25, respectively, which confirmed the diagnosis of ES. To date, no cases of ES have been reported in mainland China. The present case further emphasizes the necessity and importance of high‑resolution techniques for genetic diagnosis and for subsequent genetic counseling. The present study contributed to the phenotypic delineation of ES and confirmed the first ES patient in mainland China.

摘要

伊曼纽尔综合征(ES)是最常见的反复出现的非罗伯逊易位类型,其特征为存在众多异常。文献中已描述了100多名ES患者。该综合征的表型各异,但通常包括面部畸形、小头畸形、严重智力残疾、发育迟缓、先天性心脏病和生殖器异常。本研究描述了一名2岁男孩,患有多种畸形,包括面部畸形、严重智力残疾、生长发育迟缓、先天性心脏病、唇腭裂、生殖器畸形(小阴茎)、弱视、胸腺发育不全和听力障碍。患者的核型为47,XY,+del(22)(q13),其母亲的核型为46,XX,t(11;22)(q25;q13),9qh‑,15p+。对先证者的单核苷酸多态性阵列分析表明,22号和11号染色体分别在22q11.1‑q11.21和11q23.3‑q25处存在部分重复,这证实了ES的诊断。迄今为止,中国大陆尚未报道过ES病例。本病例进一步强调了高分辨率技术在基因诊断及后续遗传咨询中的必要性和重要性。本研究有助于对ES进行表型描述,并证实了中国大陆首例ES患者。

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