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低因子H相关5水平会导致感染引发的溶血性尿毒症综合征和膜增生性肾小球肾炎。

Low factor H-related 5 levels contribute to infection-triggered haemolytic uraemic syndrome and membranoproliferative glomerulonephritis.

作者信息

Gómez Delgado Irene, Gutiérrez-Tenorio Josué, Fraga Rodríguez Gloria M, Cavero Teresa, Arjona Emilia, Sánchez-Corral Pilar

机构信息

Complement Research Group, Hospital La Paz Institute for Health Research (IdiPAZ), La Paz University Hospital, Madrid, Spain.

Department of Immunology, Complutense University and Research Institute Hospital 12 de Octubre, Complutense University of Madrid, Madrid, Spain.

出版信息

Clin Kidney J. 2020 Mar 17;14(2):707-709. doi: 10.1093/ckj/sfaa004. eCollection 2021 Feb.

DOI:10.1093/ckj/sfaa004
PMID:35355886
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8962748/
Abstract

Dysregulation of the alternative complement pathway is a major pathogenic mechanism in two rare renal diseases: atypical haemolytic uraemic syndrome (aHUS) and membranoproliferative glomerulonephritis (MPGN). We report on a 66-year-old male with chronic hepatitis C virus (HCV) infection and a combined liver-kidney transplant that was diagnosed with MPGN at the age of 63 years and a 5-year-old boy who presented with aHUS at the age of 21 months following a infection. Both patients carried similar frameshift variants in the complement gene that segregate with reduced levels of factor H-related 5 (FHR-5). We conclude that low FHR-5 levels may predispose to viral and bacterial infections that then trigger different renal phenotypes.

摘要

补体替代途径的失调是两种罕见肾脏疾病——非典型溶血性尿毒症综合征（aHUS）和膜增生性肾小球肾炎（MPGN）的主要致病机制。我们报告了一名66岁的慢性丙型肝炎病毒（HCV）感染男性患者，其接受了肝肾联合移植，在63岁时被诊断为MPGN；还有一名5岁男孩，在感染后21个月时出现aHUS。两名患者在补体基因中携带相似的移码变异，这些变异与因子H相关蛋白5（FHR-5）水平降低相关。我们得出结论，低水平的FHR-5可能易导致病毒和细菌感染，进而引发不同的肾脏表型。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cf27/8962748/2e1af4389f08/sfaa004f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cf27/8962748/2e1af4389f08/sfaa004f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cf27/8962748/2e1af4389f08/sfaa004f1.jpg

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