Am J Med Genet C Semin Med Genet. 2013 Nov;163C(4):213-7. doi: 10.1002/ajmg.c.31383. Epub 2013 Oct 18.
Development of the craniofacial region is a remarkably complex and tightly orchestrated process. It is therefore not surprising that genetic and environmental insults frequently result in craniofacial anomalies. Nonetheless, our knowledge of their etiology and pathogenesis is still scarce, limiting our efforts at prevention. Furthermore, few standardized protocols have been developed to guide clinical and surgical interventions. In this Issue of the Seminars, reviews on the most recent research advances on craniofacial conditions, from genomics and epigenetics to ontology and medical care are discussed with emphasis on the most common anomalies of the craniofacial region: orofacial clefts, craniosynostosis, craniofacial microsomia, facial dysostosis, Robin sequence, jaw and dentition anomalies, and anterior neural tube defects. Phenotypic variability and the importance of detailed characterization using standardized terminology to better distinguish between phenotypes, new technologies (and their limitations) for genetic diagnosis, and the use of mouse models to study these conditions in both their complex phenotypic and genetic aspects are highlighted.
颅面区域的发育是一个非常复杂和协调的过程。因此,遗传和环境因素的干扰经常导致颅面异常也就不足为奇了。尽管如此,我们对其病因和发病机制的了解仍然很少,这限制了我们的预防工作。此外,很少有标准化的方案来指导临床和手术干预。在本期《研讨会》中,对颅面疾病的最新研究进展进行了综述,从基因组学和表观遗传学到本体论和医疗保健,重点讨论了颅面区域最常见的异常:口腔颌面裂、颅缝早闭、颅面小颌畸形、面骨发育不全、Robin 序列、颌骨和牙齿异常以及前神经管缺陷。表型变异性以及使用标准化术语进行详细特征描述的重要性,以便更好地区分表型、用于基因诊断的新技术(及其局限性),以及使用小鼠模型研究这些疾病在复杂表型和遗传方面的重要性,都得到了强调。