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探讨先天性脊柱畸形与神经管缺陷之间的关联。

Exploring the association between congenital vertebral malformations and neural tube defects.

机构信息

Department of Orthopedic Surgery, Guangdong Provincial People's Hospital (Guangdong Academy of Medical Sciences), Southern Medical University, Guangzhou, Guangdong, China.

Department of Orthopaedics and Traumatology, School of Clinical Medicine, Li Ka Shing Faculty of Medicine, The University of Hong Kong, Hong Kong, China.

出版信息

J Med Genet. 2023 Nov 27;60(12):1146-1152. doi: 10.1136/jmg-2023-109501.

Abstract

Congenital vertebral malformations (CVMs) and neural tube defects (NTDs) are common birth defects affecting the spine and nervous system, respectively, due to defects in somitogenesis and neurulation. Somitogenesis and neurulation rely on factors secreted from neighbouring tissues and the integrity of the axial structure. Crucial signalling pathways like Wnt, Notch and planar cell polarity regulate somitogenesis and neurulation with significant crosstalk. While previous studies suggest an association between CVMs and NTDs, the exact mechanism underlying this relationship remains unclear. In this review, we explore embryonic development, signalling pathways and clinical phenotypes involved in the association between CVMs and NTDs. Moreover, we provide a summary of syndromes that exhibit occurrences of both CVMs and NTDs. We aim to provide insights into the potential mechanisms underlying the association between CVMs and NTDs, thereby facilitating clinical diagnosis and management of these anomalies.

摘要

先天性脊柱畸形(CVMs)和神经管缺陷(NTDs)分别是由于体节形成和神经管闭合缺陷而影响脊柱和神经系统的常见出生缺陷。体节形成和神经管闭合依赖于来自邻近组织的分泌因子和轴性结构的完整性。Wnt、Notch 和平面细胞极性等关键信号通路通过显著的串扰调节体节形成和神经管闭合。虽然之前的研究表明 CVMs 和 NTDs 之间存在关联,但这种关系的确切机制仍不清楚。在这篇综述中,我们探讨了胚胎发育、信号通路和参与 CVMs 和 NTDs 关联的临床表型。此外,我们还总结了同时表现出 CVMs 和 NTDs 的综合征。我们的目的是深入了解 CVMs 和 NTDs 之间关联的潜在机制,从而促进这些异常的临床诊断和管理。

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