由 CUBN 基因突变引起的孤立性蛋白尿:病例报告和文献综述。

IF 0.7 Q4 UROLOGY & NEPHROLOGY Case Reports in Nephrology and Dialysis Pub Date : 2023-05-26 eCollection Date: 2023-01-01 DOI:10.1159/000530466
Jingyang Ran, Qingsong Chen, Yudong Hu, Pengfei Yang, Guiquan Yu, Xiaohui Liao, Jianrong Lei
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引用次数: 0

摘要

立方体蛋白(CUBN)基因突变通常会导致 Imerslund-Gräsbeck 综合征,而由 CUBN 变异导致的孤立性蛋白尿却鲜有报道。临床表现主要是非肾病范围的慢性孤立性蛋白尿。然而,迄今为止的研究结果表明,与 CUBN 基因异常相关的孤立性蛋白尿是良性的,不会影响肾功能的长期预后。我们发现了两名因复合杂合子 CUBN 基因突变而引发孤立性蛋白尿的患者。这两名患者的肾功能在长达10年的随访期间均保持正常,证明了CUBN基因变异引起的蛋白尿是良性的。该研究发现了两个新的突变位点,扩大了CUBN变异的基因型谱。此外,还对该病的病因、发病机制、临床表现、辅助检查和治疗进行了综述,旨在为临床治疗提供进一步指导。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

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Isolated Proteinuria Caused by CUBN Gene Mutations: A Case Report and Review of the Literature.

Mutations in the cubilin (CUBN) gene commonly cause Imerslund-Gräsbeck syndrome, while isolated proteinuria as a result of CUBN variations is rarely reported. The clinical manifestation is mainly chronic isolated proteinuria in the non-nephrotic range. However, findings to date suggest that isolated proteinuria associated with abnormalities in the CUBN gene is benign and does not affect long-term prognosis of kidney function. We identified 2 patients with isolated proteinuria triggered by compound heterozygous CUBN mutations. Renal functions of both patients remained normal over a 10-year follow-up period, supporting the benign nature of proteinuria caused by CUBN gene variations. Two novel mutation sites were detected, expanding the genotypic spectrum of CUBN variations. In addition, etiology, pathogenesis, clinical manifestations, auxiliary examination, and treatment of the condition were reviewed, with the aim of providing further guidance for clinical management.

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来源期刊
CiteScore
1.20
自引率
0.00%
发文量
36
审稿时长
10 weeks
期刊介绍: This peer-reviewed online-only journal publishes original case reports covering the entire spectrum of nephrology and dialysis, including genetic susceptibility, clinical presentation, diagnosis, treatment or prevention, toxicities of therapy, critical care, supportive care, quality-of-life and survival issues. The journal will also accept case reports dealing with the use of novel technologies, both in the arena of diagnosis and treatment. Supplementary material is welcomed.
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