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VACTERL与半侧颜面短小畸形之间的重叠,说明了在轴旁中胚层发育异常综合征(AMDC)中所见的一系列畸形。

Overlap between VACTERL and hemifacial microsomia illustrating a spectrum of malformations seen in axial mesodermal dysplasia complex (AMDC).

作者信息

Bergmann Carsten, Zerres Klaus, Peschgens Thomas, Senderek Jan, Hörnchen Helmut, Rudnik-Schöneborn Sabine

机构信息

Institute of Human Genetics, Aachen University of Technology, Germany.

出版信息

Am J Med Genet A. 2003 Aug 30;121A(2):151-5. doi: 10.1002/ajmg.a.20167.

Abstract

We describe a male infant born to consanguineous healthy parents with multiple congenital anomalies of the skeleton and internal organs. His phenotype displays an overlap between VACTERL and hemifacial microsomia (oculo-auriculo-vertebral spectrum/OAV). In addition, striking asymmetry of the malformations further supports the classification as part of the "axial mesodermal dysplasia complex" (AMDC) which is supposed to arise from disturbed mesodermal cell migration during early blastogenesis. The present case was instructive to review the continuous spectrum of AMDC related anomalies.

摘要

我们描述了一名男婴,其父母为近亲且健康,该男婴患有骨骼和内脏的多种先天性异常。他的表型显示VACTERL和半侧颜面短小畸形(眼-耳-脊椎谱系/OAV)之间存在重叠。此外,畸形的明显不对称进一步支持将其归类为“轴旁中胚层发育异常复合体”(AMDC)的一部分,该复合体被认为是由于早期胚泡形成过程中中胚层细胞迁移紊乱所致。本病例对于回顾与AMDC相关的异常的连续谱系具有指导意义。

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