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伴有PTPN11基因突变的豹皮综合征,表现出豹皮综合征的六种主要症状。

LEOPARD Syndrome with PTPN11 Gene Mutation Showing Six Cardinal Symptoms of LEOPARD.

作者信息

Kim Jihyun, Kim Mi Ri, Kim Hee Jung, Lee Kyung-A, Lee Min-Geol

机构信息

Department of Dermatology and Cutaneous Biology Research Institute, Yonsei University College of Medicine, Seoul, Korea.

出版信息

Ann Dermatol. 2011 May;23(2):232-5. doi: 10.5021/ad.2011.23.2.232. Epub 2011 May 27.

Abstract

LEOPARD multiple congenital anomaly syndrome inherited in an autosomal dominant manner. LEOPARD is an acronym for Lentigines, Eletrocardiographic conduction defects, Ocular hypertelorism, Pulmonary valve stenosis, Abnormalities of the genitalia, Retardation of growth, and Deafness. Clinical diagnosis is primarily based on multiple lentigines, typical facial features, and the presence of hypertrophic cardiomyopathy and/or café-au-lait macules. We report a typical case of LEOPARD syndrome with PTPN11 gene mutation associated with lentigines, electrocardiograph abnormality, ocular hypertelorism, pulmonary valve stenosis, growth retardation, and sensorineural hearing loss.

摘要

豹皮综合征以常染色体显性方式遗传。LEOPARD是雀斑、心电图传导缺陷、眼距过宽、肺动脉瓣狭窄、生殖器异常、生长发育迟缓及耳聋的英文首字母缩写。临床诊断主要基于多发性雀斑、典型面部特征以及肥厚型心肌病和/或牛奶咖啡斑的存在。我们报告了一例典型的豹皮综合征病例,其PTPN11基因突变,伴有雀斑、心电图异常、眼距过宽、肺动脉瓣狭窄、生长发育迟缓及感音神经性听力损失。

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