一个被忽视的家庭,患有罕见的法布里病突变。

IF 0.6
Gamze Babur Güler, Arda Güler, Abdullah Doğan, Zümrüt Arslan Gülten, Aysel Türkvatan Cansever, Mehmet Karacan
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引用次数: 0

摘要

法布里病是一种罕见的疾病,其特征是多器官受累,由GLA基因突变引起。尽管已经在GLA基因中发现了1000多个突变,但新突变的发现和检测仍在继续增强该数据集。我们报告一个在我们诊所检查心脏瓣膜疾病的病人,他有肾移植和血液透析的历史。在临床高度怀疑的情况下,我们诊断出法布里病,并将讨论其对家庭的重大影响。有效的家庭筛查使我们能够识别未受影响的家庭成员,从而预防或减轻未来潜在的器官受累。此外,在该家族中发现的突变,虽然以前很少报道,但在一些来源中仍被描述为不确定意义变异(VUS)。我们相信这一发现将对遗传文献做出有价值的贡献。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
An Overlooked Family with a Rare Mutation for Fabry Disease.

Fabry disease is a rare disorder characterized by multi-organ involvement, caused by mutations in the GLA gene. Although more than 1,000 mutations have been identified in the GLA gene, the discovery and detection of new mutations continue to enhance this dataset. We report a patient examined at our clinic for heart valve disease, who had a history of kidney transplantation and hemodialysis. With a high clinical suspicion, we diagnosed Fabry disease and will discuss its significant impact on the family. Effective family screening allowed us to identify unaffected family members, thus preventing or mitigating potential future organ involvement. Additionally, the mutation found in this family, although rarely reported before, is still described as a variant of uncertain significance (VUS) in some sources. We believe this finding will make a valuable contribution to genetic literature.

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