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米歇尔综合征、卡内瓦莱综合征、阻塞性睡眠呼吸暂停综合征和马尔皮克综合征:单一疾病(3MC综合征)的可变表现?

Michels syndrome, Carnevale syndrome, OSA syndrome, and Malpuech syndrome: variable expression of a single disorder (3MC syndrome)?

作者信息

Titomanlio Luigi, Bennaceur Selim, Bremond-Gignac Dominique, Baumann Clarisse, Dupuy Olivier, Verloes Alain

机构信息

Clinical Genetics Unit, Robert Debré Hospital, Paris, France.

出版信息

Am J Med Genet A. 2005 Sep 1;137A(3):332-5. doi: 10.1002/ajmg.a.30878.

Abstract

We report on a 3-year-old girl with Michels syndrome, a rare condition characterized by craniosynostosis, blepharophimosis, ptosis, epicanthus inversus, cleft lip/palate, abnormal supra-umbilical abdominal wall, and mental deficiency. The phenotypic findings are compared with the six previously reported Michels cases, and with patients referred to as Carnevale, OSA, and Malpuech syndromes. Michels syndrome is characterized by cleft lip and palate, anterior chamber anomalies, blepharophimosis, epicanthus inversus, and craniosynostosis. Carnevale syndrome shows hypertelorism, downslanting palpebral fissures, ptosis, strabismus synophrys, large and fleshy ears, and lozenge-shaped diastasis around the umbilicus. OSA syndrome resembles Carnevale, with humeroradial synostoses, and spinal anomalies as extra features. Malpuech syndrome shows IUGR, hypertelorism, cleft lip and palate, micropenis, hypospadias, renal anomalies, and caudal appendage. All are autosomal recessive. Despite the presence of apparently distinctive key features, it appears that these four entities share multiple similarities in the facial Gestalt and the pattern of MCA. Those similarities lead us to postulate that they belong to the same spectrum, which could be referred to as "3MC syndrome" (Malpuech-Michels-Mingarelli-Carnevale syndrome).

摘要

我们报告了一名患有米歇尔综合征的3岁女孩,这是一种罕见的病症,其特征为颅缝早闭、睑裂狭小、上睑下垂、内眦赘皮、唇腭裂、脐上腹壁异常和智力缺陷。将该病例的表型特征与之前报道的6例米歇尔综合征病例以及被称为卡内瓦莱综合征、OSA综合征和马尔普埃什综合征的患者进行了比较。米歇尔综合征的特征为唇腭裂、前房异常、睑裂狭小、内眦赘皮和颅缝早闭。卡内瓦莱综合征表现为眼距增宽、睑裂向下倾斜、上睑下垂、斜视、连眉、耳朵大且肥厚,以及脐周菱形皮肤缺损。OSA综合征与卡内瓦莱综合征相似,额外特征为肱桡关节融合和脊柱异常。马尔普埃什综合征表现为宫内生长受限、眼距增宽、唇腭裂、小阴茎、尿道下裂、肾脏异常和尾状附属物。所有这些综合征均为常染色体隐性遗传。尽管存在明显独特的关键特征,但这四种病症在面部形态和大脑中动脉模式上似乎有多个相似之处。这些相似之处使我们推测它们属于同一谱系,可称为“3MC综合征”(马尔普埃什 - 米歇尔 - 明加雷利 - 卡内瓦莱综合征)。

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