Genetic and Metabolic Central Laboratory, Maternal and Child Health Hospital of Guangxi Zhuang Autonomous Region, Nanning, Guangxi, PR China; Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, PR China.
Genetic and Metabolic Central Laboratory, Maternal and Child Health Hospital of Guangxi Zhuang Autonomous Region, Nanning, Guangxi, PR China.
Clin Chim Acta. 2017 Nov;474:159-164. doi: 10.1016/j.cca.2017.09.022. Epub 2017 Sep 29.
3-M syndrome is a clinically recognizable yet under-diagnosed primordial growth retardation disorder. Molecular testing for CUL7, OBSL1 or CCDC8 genes can provide confirmed diagnosis for patients at prenatal or early age. So far, the clinical and molecular features of Chinese 3-M syndrome patients have not been reported.
In this article, the authors performed prenatal and early diagnosis of Chinese patients with 3-M syndrome by Next-Generation Sequencing.
The authors reported six unrelated Chinese 3-M syndrome patients. Five of the six patients were diagnosed before two years of age including one prenatal case. The authors identified six novel pathogenic variants and five previously reported pathogenic variants. The authors' clinical evaluations indicated that Chinese 3-M syndrome patients share similar recognizable features as those reported in patients of other ethnic background. The authors noticed some uncommon features in this small cohort of Chinese patients such as delayed motor development at early ages, undelayed bone age and presence of lower eyelid fat pads.
The authors' study of Chinese 3-M syndrome patients revealed novel mutations and clinical phenotypes.
3-M 综合征是一种临床可识别但诊断不足的原始生长发育迟缓障碍。CUL7、OBSL1 或 CCDC8 基因的分子检测可为产前或早期的患者提供明确诊断。到目前为止,尚未报道中国 3-M 综合征患者的临床和分子特征。
在本文中,作者通过下一代测序对中国 3-M 综合征患者进行了产前和早期诊断。
作者报道了 6 例无关的中国 3-M 综合征患者。其中 5 例在 2 岁之前被诊断,包括 1 例产前病例。作者发现了 6 个新的致病性变异和 5 个先前报道的致病性变异。作者的临床评估表明,中国 3-M 综合征患者与其他种族背景的患者报告的相似的可识别特征相似。作者注意到这一小部分中国患者存在一些不常见的特征,如早期运动发育迟缓、骨龄不延迟和下眼睑脂肪垫存在。
作者对中国 3-M 综合征患者的研究揭示了新的突变和临床表型。