Xie Xiaoying, Du Juan, Geng Shunkang, Yi Baoqin, Li Qingpu, Zuo Jiangcheng
Department of Clinical Laboratory, Yichang Yiling People's Hospital, Yichang, Hubei, 443100, China.
Hereditas. 2024 Feb 20;161(1):9. doi: 10.1186/s41065-024-00313-3.
Congenital fibrinogen disorders are a group of coagulation deficiencies caused by fibrinogen defects and are divided into four types, including afibrinogenemia, hypofibrinogenemia, dysfibrinogenemia, and hypodysfibrinogenemia. In this study, we collected a family with hypofibrinogenemia, and genetics analysis identify a novel pathogenic variants (c.668G > C, p.Arg223Thr) in the FGG gene. And electron microscope observation revealed significant changes in the ultrastructure of fibrin of the proband. Our research expands the phenotypic and genetic spectrum associated with the FGG gene, which would facilitate in genetic counselling and prenatal genetic diagnosis.
先天性纤维蛋白原异常是一组由纤维蛋白原缺陷引起的凝血功能缺陷,分为四种类型,包括无纤维蛋白原血症、低纤维蛋白原血症、异常纤维蛋白原血症和低异常纤维蛋白原血症。在本研究中,我们收集了一个低纤维蛋白原血症家族,基因分析在FGG基因中鉴定出一个新的致病变异(c.668G>C,p.Arg223Thr)。电子显微镜观察显示先证者纤维蛋白超微结构有显著变化。我们的研究扩展了与FGG基因相关的表型和遗传谱,这将有助于遗传咨询和产前基因诊断。