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里基耶里-科斯塔和佩雷拉综合征:两例巴西同胞兄妹的报告并文献复习。

The Richieri-Costa and Pereira syndrome: report of two Brazilian siblings and review of literature.

机构信息

Assistance Center for Cleft Lip and Palate, CAIF, Curitiba, Paraná, Brazil.

出版信息

Am J Med Genet A. 2011 May;155A(5):1173-7. doi: 10.1002/ajmg.a.33975. Epub 2011 Apr 11.

Abstract

Richieri-Costa and Pereira syndrome is a rare autosomal recessive disorder characterized specially by Pierre Robin sequence with cleft mandible and limb anomalies. There are a typical laryngeal anomaly which encompass short and round larynx, absent or abnormal epiglottis, and abnormal aryepiglottic folds. Most patients reported were from Brazil. We describe a brother and sister with Richieri-Costa and Pereira syndrome on another Brazilian family documenting their physical findings and laryngeal defects. We also review the literature and discuss the main clinical characteristics and etiology.

摘要

里谢尔-佩雷拉综合征是一种罕见的常染色体隐性遗传疾病,其特征特别表现为小下颌伴有腭裂和肢体畸形的 Pierre Robin 序列。存在典型的喉部异常,包括短而圆的喉部、缺失或异常的会厌以及异常的杓会厌皱襞。大多数报道的患者来自巴西。我们描述了另一个巴西家庭中的一对兄妹患有里谢尔-佩雷拉综合征,记录了他们的体格检查结果和喉部缺陷。我们还回顾了文献并讨论了主要的临床特征和病因。

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