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多发性翼状胬肉综合征:类似特纳综合征的表现。

Multiple pterygium syndrome: mimicking the findings of Turner syndrome.

作者信息

Güven Ayla, Kirmizibekmez Heves

机构信息

Clinics of Pediatric Endocrinology, Göztepe Educational and Research Hospital, Dr. Erkin Caddesi Eğitim, Kadiköy, Istanbul, Turkey.

出版信息

J Pediatr Endocrinol Metab. 2011;24(11-12):1089-93.

Abstract

Multiple pterygium syndrome (MPS or Escobar syndrome) is a rare, generally autosomal recessive disorder characterized by multiple congenital joint contractures and multiple skin webs. An 11.5-year-old girl with a working diagnosis of Turner syndrome (TS) was referred for her phenotypic features and growth retardation. Pterygium of the neck, low posterior hairline, widely spaced nipples, cubitus valgus, upslanting palpebral fissures, hypertelorism, micrognathia, low-set ears, downturning corners of the mouth, long philtrum, high-arched palate, digital and intercrural webbings, and aplasia of the labia majora were indicative of MPS (Escobar syndrome). Her mental status was normal. Facial asymmetry was present due to cervical webs. Normal karyotype, gonadal functions, and cardiac and urinary system findings helped in excluding TS. Genetic diseases associated with skin webs were revised in differential diagnosis.

摘要

多发性翼状胬肉综合征(MPS或埃斯科瓦尔综合征)是一种罕见的、通常为常染色体隐性遗传的疾病,其特征为多发性先天性关节挛缩和多处皮肤蹼。一名11.5岁、初步诊断为特纳综合征(TS)的女孩因表型特征和生长发育迟缓前来就诊。颈部翼状胬肉、低后发际线、乳头间距宽、肘外翻、睑裂上斜、眼距增宽、小颌畸形、低位耳、嘴角下垂、人中长、高拱腭、手指和股间蹼以及大阴唇发育不全提示为MPS(埃斯科瓦尔综合征)。她的精神状态正常。由于颈部蹼导致面部不对称。正常的核型、性腺功能以及心脏和泌尿系统检查结果有助于排除TS。在鉴别诊断中对与皮肤蹼相关的遗传性疾病进行了重新评估。

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