| Literature DB >> 30986546 |
Chun Yi Ting1, Neha Singh Bhatia2, Jiin Ying Lim3, Chew-Yin Jasmine Goh4, Rashida Farhad Vasanwala5, Caroline Choo-Phaik Ong6, Wan Tew Seow7, Vincent Kok-Leng Yeow8, Teck Wah Ting9, Ivy Swee-Lian Ng10, Saumya Shekhar Jamuar11.
Abstract
Meier-Gorlin syndrome (MGS) is a rare autosomal recessive disorder characterized by the triad of short stature, microtia and absent or small patellae. We report on a patient with MGS secondary to biallelic mutations in CDC45 detected on whole exome sequencing (WES). Patients with MGS caused by mutations in CDC45 display a distinct phenotype characterized by craniosynostosis and anorectal malformation. Our patient had craniosynostosis, anorectal malformation and short stature, but did not have the microtia or patella hypoplasia. Our report also highlights the value of WES in aiding diagnosis of patients with rare genetic diseases. In conclusion, our case report and review of the literature illustrates the unique features of CDC45-related MGS as well as the benefits of WES in reducing the diagnostic odyssey for patients with rare genetic disorders.Entities:
Keywords: Craniosynostosis; Diagnostic odyssey; Meier-Gorlin syndrome; anorectal malformations; whole exome sequencing
Year: 2019 PMID: 30986546 DOI: 10.1016/j.ejmg.2019.04.009
Source DB: PubMed Journal: Eur J Med Genet ISSN: 1769-7212 Impact factor: 2.708