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[下颌面骨发育不全或弗朗西谢蒂-茨瓦伦-克莱因综合征:附2例报告]

[Mandibulofacial dysostosis or Franceschetti-Zwahlen-Klein syndrome: apropos of 2 cases].

作者信息

Donadio P, Bolla G, Donadio G

机构信息

Clinica S. Stefano, Napoli, Italia.

出版信息

Pediatr Med Chir. 1989 May-Jun;11(3):355-7.

PMID:2594569
Abstract

The Authors report two cases of mandibulofacial dysostosis: a three-months-old girl who presented with palpebral fissures in an antimongoloid direction, cleft palate, coloboma of the lower lid, hypoplasia of the malar bones and mandible, malformation of the external ears, two clefts between the mouth and left ear, minimal naso-frontal angle and a one-month-old boy who presented with similar structural deformities. These features can be considered characteristic of mandibulofacial dysostosis, whose most common finding is the presence of bilateral signs.

摘要

作者报告了两例下颌面骨发育不全病例

一名三个月大的女孩,表现为睑裂呈反蒙古人种方向、腭裂、下睑缺损、颧骨和下颌骨发育不全、外耳畸形、口角与左耳之间有两条裂隙、鼻额角极小;以及一名一个月大的男孩,也表现出类似的结构畸形。这些特征可被视为下颌面骨发育不全的典型表现,其最常见的表现是双侧体征。

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