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具有复杂临床表现的日本17q12缺失综合征

Japanese 17q12 Deletion Syndrome with Complex Clinical Manifestations.

作者信息

Hasegawa Yutaka, Takahashi Yoshihiko, Nagasawa Kan, Kinno Hirofumi, Oda Tomoyasu, Hangai Mari, Odashima Yoshimi, Suzuki Yoko, Shimizu Jun, Ando Toshihiko, Egawa Isao, Hashizume Kouhei, Nata Koji, Yabe Daisuke, Horikawa Yukio, Ishigaki Yasushi

机构信息

Division of Diabetes, Metabolism and Endocrinology, Department of Internal Medicine, Iwate Medical University, Japan.

Department of Ophthalmology, School of Medicine, Iwate Medical University, Japan.

出版信息

Intern Med. 2024;63(5):687-692. doi: 10.2169/internalmedicine.1660-23.

Abstract

17q12 deletion syndrome is a rare chromosomal anomaly with variable phenotypes, caused by the heterozygous deletion of chromosome 17q12. We herein report a 35-year-old Japanese patient with chromosomal 17q12 deletion syndrome identified by de novo deletion of the 1.46 Mb segment at the 17q12 band by genetic analyses. He exhibited a wide range of phenotypes, such as maturity-onset diabetes of the young (MODY) type 5, structural or functional abnormalities of the kidney, liver, and pancreas; facial dysmorphic features, electrolyte disorders; keratoconus, and acquired perforating dermatosis. This case report provides valuable resources concerning the clinical spectrum of rare 17q12 deletion syndrome.

摘要

17q12缺失综合征是一种罕见的染色体异常疾病,具有多种可变表型,由17号染色体q12区域的杂合缺失引起。我们在此报告一名35岁的日本患者,通过基因分析发现其17q12带发生了1.46 Mb片段的新发缺失,从而确诊为染色体17q12缺失综合征。他表现出多种表型,如青年发病型成年糖尿病(MODY)5型、肾脏、肝脏和胰腺的结构或功能异常、面部畸形特征、电解质紊乱、圆锥角膜和获得性穿通性皮肤病。本病例报告为罕见的17q12缺失综合征的临床谱提供了有价值的资料。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1c84/10982014/4d82f90ab77d/1349-7235-63-0687-g001.jpg

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