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[赛特勒-乔岑综合征:一例报告]

[Saethre-Chotzen syndrome: a case report].

作者信息

Díez de Los Ríos Quintanero Blanca, Gracia Rojas Eva, Ortiz Movilla Roberto, Cabrejas Núñez María J, Marín Gabriel Miguel Á

机构信息

Servicio de Pediatría, Hospital Universitario Puerta de Hierro-Majadahonda, Madrid, España.

Unidad de Neonatología, Servicio de Pediatría, Hospital Universitario Puerta de Hierro-Majadahonda, Madrid, España.

出版信息

Arch Argent Pediatr. 2021 Apr;119(2):e129-e132. doi: 10.5546/aap.2021.e129.

Abstract

The Saethre-Chotzen syndrome is a craniofacial malformation syndrome characterized by synostosis of coronal sutures and limb anomalies. The estimated prevalence of this syndrome is 1 in 25 000-50 000 live births. We present a case report of a neonate, without relevant family history, who presented craniofacial alterations at birth. Given the phenotypic features, a cranial computed tomography scan was performed, showing partial fusion of the coronal suture, evidencing the presence Síndrome de Saethre-Chotzen: a propósito de un caso Saethre-Chotzen syndrome: a case report of wormian bones in the metopic and right lambdoid location. With the clinical suspicion of craniofacial malformation syndrome, an analysis of the directed exome was requested confirming that the patient is a heterozygous carrier of the pathogenic variant c.415C>A, which induces a change of proline to threonine at position 139 of the TWIST1 gene, responsible for Saethre-Chotzen syndrome. The presence of wormian bones, a finding not described so far in the literature, extends the well-known phenotypic variability of this syndrome.

摘要

塞特勒-乔岑综合征是一种颅面畸形综合征,其特征为冠状缝早闭和肢体异常。该综合征的估计患病率为每25000 - 50000例活产中有1例。我们报告一例新生儿病例,该患儿无相关家族史,出生时即出现颅面改变。鉴于其表型特征,进行了头颅计算机断层扫描,显示冠状缝部分融合,在额缝和右侧人字缝位置发现了缝间骨,证实存在塞特勒-乔岑综合征。因临床怀疑为颅面畸形综合征,遂进行了定向外显子组分析,确认该患者是致病性变异c.415C>A的杂合携带者,该变异导致TWIST1基因第139位的脯氨酸变为苏氨酸,而TWIST1基因是导致塞特勒-乔岑综合征的原因。缝间骨的存在是文献中迄今未描述的发现,这扩展了该综合征众所周知的表型变异性。

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