Low factor H-related 5 levels contribute to infection-triggered haemolytic uraemic syndrome and membranoproliferative glomerulonephritis
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 combin...
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| Published in: | Clinical kidney journal Vol. 14; no. 2; pp. 707 - 709 |
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| Main Authors: | , , , , , |
| Format: | Journal Article |
| Language: | English |
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England
Oxford University Press
01-02-2021
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| 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. |
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| AbstractList | 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. Keywords: CFHR5, complement, HCV, HUS, MPGN, Streptococcus, pneumoniae 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. 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. 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. |
| Audience | Academic |
| Author | Gómez Delgado, Irene Arjona, Emilia Gutiérrez-Tenorio, Josué Fraga Rodríguez, Gloria M Cavero, Teresa Sánchez-Corral, Pilar |
| AuthorAffiliation | 4 Department of Nephrology, Hospital Universitario 12 de Octubre , Madrid, Spain 2 Department of Immunology, Complutense University and Research Institute Hospital 12 de Octubre, Complutense University of Madrid , Madrid, Spain 1 Complement Research Group, Hospital La Paz Institute for Health Research (IdiPAZ), La Paz University Hospital , Madrid, Spain 6 Centre for Biomedical Network Research on Rare Diseases (CIBERER) , Madrid, Spain 5 Department of Molecular Biomedicine, Centre for Biological Research , Madrid, Spain 3 Department of Paediatric Nephrology, Hospital de la Santa Creu i Sant Pau, Universitat Autònoma de Barcelona , Barcelona, Spain |
| AuthorAffiliation_xml | – name: 2 Department of Immunology, Complutense University and Research Institute Hospital 12 de Octubre, Complutense University of Madrid , Madrid, Spain – name: 5 Department of Molecular Biomedicine, Centre for Biological Research , Madrid, Spain – name: 6 Centre for Biomedical Network Research on Rare Diseases (CIBERER) , Madrid, Spain – name: 4 Department of Nephrology, Hospital Universitario 12 de Octubre , Madrid, Spain – name: 3 Department of Paediatric Nephrology, Hospital de la Santa Creu i Sant Pau, Universitat Autònoma de Barcelona , Barcelona, Spain – name: 1 Complement Research Group, Hospital La Paz Institute for Health Research (IdiPAZ), La Paz University Hospital , Madrid, Spain |
| Author_xml | – sequence: 1 givenname: Irene surname: Gómez Delgado fullname: Gómez Delgado, Irene organization: Complement Research Group, Hospital La Paz Institute for Health Research (IdiPAZ), La Paz University Hospital, Madrid, Spain – sequence: 2 givenname: Josué surname: Gutiérrez-Tenorio fullname: Gutiérrez-Tenorio, Josué organization: Department of Immunology, Complutense University and Research Institute Hospital 12 de Octubre, Complutense University of Madrid, Madrid, Spain – sequence: 3 givenname: Gloria M surname: Fraga Rodríguez fullname: Fraga Rodríguez, Gloria M organization: Department of Paediatric Nephrology, Hospital de la Santa Creu i Sant Pau, Universitat Autònoma de Barcelona, Barcelona, Spain – sequence: 4 givenname: Teresa surname: Cavero fullname: Cavero, Teresa organization: Department of Nephrology, Hospital Universitario 12 de Octubre, Madrid, Spain – sequence: 5 givenname: Emilia surname: Arjona fullname: Arjona, Emilia organization: Centre for Biomedical Network Research on Rare Diseases (CIBERER), Madrid, Spain – sequence: 6 givenname: Pilar orcidid: 0000-0003-4212-1233 surname: Sánchez-Corral fullname: Sánchez-Corral, Pilar organization: Centre for Biomedical Network Research on Rare Diseases (CIBERER), Madrid, Spain |
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| Keywords | Streptococcus HUS pneumoniae CFHR5 HCV complement MPGN |
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| License | The Author(s) 2020. Published by Oxford University Press on behalf of ERA-EDTA. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com |
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| SubjectTerms | Bacterial pneumonia Exceptional Cases Glomerulonephritis Health aspects Hepatitis C virus Kidneys Liver Pneumonia Transplantation |
| Title | Low factor H-related 5 levels contribute to infection-triggered haemolytic uraemic syndrome and membranoproliferative glomerulonephritis |
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