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

Irene Gómez Delgado | Zendy; Josué Gutiérrez-Tenorio | Zendy; Gloria Maria Fraga Rodríguez | Zendy; Teresa Cavero | Zendy; Emilia Arjona | Zendy; Pilar SánchezCorral | Zendy

Open Access

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

Author(s) -

Irene Gómez Delgado,

Josué Gutiérrez-Tenorio,

Gloria Maria Fraga Rodríguez,

Teresa Cavero,

Emilia Arjona,

Pilar SánchezCorral

Publication year - 2020

Publication title -

clinical kidney journal

Language(s) - English

Resource type - Journals

eISSN - 2048-8513

pISSN - 2048-8505

DOI - 10.1093/ckj/sfaa004

Subject(s) - membranoproliferative glomerulonephritis , atypical hemolytic uremic syndrome , immunology , medicine , alternative complement pathway , glomerulonephritis , complement factor b , factor h , complement factor i , complement system , kidney , antibody

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.

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