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

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 Streptococcus pneumoniae infection. Both patients carried similar frameshift variants in the complement CFHR5 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.

SUBMITTER: Gomez Delgado I

PROVIDER: S-EPMC8962748 | biostudies-literature | 2021 Feb

REPOSITORIES: biostudies-literature

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

Gómez Delgado Irene I Gutiérrez-Tenorio Josué J Fraga Rodríguez Gloria M GM Cavero Teresa T Arjona Emilia E Sánchez-Corral Pilar P

Clinical kidney journal 20200317 2

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 <i>Streptococcus pneumoniae</i> infectio ...[more]

PMID: 35355886

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Eculizumab in secondary atypical haemolytic uraemic syndrome.

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| S-EPMC5410989 | biostudies-literature

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