{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,2,21]],"date-time":"2025-02-21T19:33:01Z","timestamp":1740166381414,"version":"3.37.3"},"reference-count":5,"publisher":"Oxford University Press (OUP)","issue":"2","license":[{"start":{"date-parts":[[2020,3,17]],"date-time":"2020-03-17T00:00:00Z","timestamp":1584403200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"name":"Spanish \u2018Instituto de Salud Carlos III"},{"DOI":"10.13039\/501100008530","name":"European Regional Development Fund","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100008530","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000780","name":"European Union","doi-asserted-by":"publisher","award":["PI16\/00723"],"award-info":[{"award-number":["PI16\/00723"]}],"id":[{"id":"10.13039\/501100000780","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Autonomous Region of Madrid","award":["B2017\/BMD-3673"],"award-info":[{"award-number":["B2017\/BMD-3673"]}]},{"DOI":"10.13039\/501100003329","name":"Spanish \u2018Ministerio de Econom\u00eda y Competitividad\u2019","doi-asserted-by":"crossref","award":["SAF2014-52339P","BES-2015-073833"],"award-info":[{"award-number":["SAF2014-52339P","BES-2015-073833"]}],"id":[{"id":"10.13039\/501100003329","id-type":"DOI","asserted-by":"crossref"}]},{"name":"Autonomous Region of Madrid","award":["B2017\/BMD-3673"],"award-info":[{"award-number":["B2017\/BMD-3673"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2021,2,16]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>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\u2013kidney 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\u2009months following a Streptococcus pneumoniae infection. Both patients carried similar frameshift variants in the complement CFHR5 gene that segregate with reduced levels of factor H\u2013related 5 (FHR-5). We conclude that low FHR-5 levels may predispose to viral and bacterial infections that then trigger different renal phenotypes.<\/jats:p>","DOI":"10.1093\/ckj\/sfaa004","type":"journal-article","created":{"date-parts":[[2020,1,13]],"date-time":"2020-01-13T12:08:14Z","timestamp":1578917294000},"page":"707-709","source":"Crossref","is-referenced-by-count":3,"title":["Low factor H-related 5 levels contribute to infection-triggered haemolytic uraemic syndrome and membranoproliferative glomerulonephritis"],"prefix":"10.1093","volume":"14","author":[{"given":"Irene","family":"G\u00f3mez Delgado","sequence":"first","affiliation":[{"name":"Complement Research Group, Hospital La Paz Institute for Health Research (IdiPAZ), La Paz University Hospital, Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Josu\u00e9","family":"Guti\u00e9rrez-Tenorio","sequence":"additional","affiliation":[{"name":"Department of Immunology, Complutense University and Research Institute Hospital 12 de Octubre, Complutense University of Madrid, Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gloria M","family":"Fraga Rodr\u00edguez","sequence":"additional","affiliation":[{"name":"Department of Paediatric Nephrology, Hospital de la Santa Creu i Sant Pau, Universitat Aut\u00f2noma de Barcelona, Barcelona, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Teresa","family":"Cavero","sequence":"additional","affiliation":[{"name":"Department of Nephrology, Hospital Universitario 12 de Octubre, Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Emilia","family":"Arjona","sequence":"additional","affiliation":[{"name":"Department of Molecular Biomedicine, Centre for Biological Research, Madrid, Spain"},{"name":"Centre for Biomedical Network Research on Rare Diseases (CIBERER), Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4212-1233","authenticated-orcid":false,"given":"Pilar","family":"S\u00e1nchez-Corral","sequence":"additional","affiliation":[{"name":"Complement Research Group, Hospital La Paz Institute for Health Research (IdiPAZ), La Paz University Hospital, Madrid, Spain"},{"name":"Centre for Biomedical Network Research on Rare Diseases (CIBERER), Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2020,3,17]]},"reference":[{"key":"2021021623390719700_sfaa004-B1","doi-asserted-by":"crossref","first-page":"400","DOI":"10.1016\/j.mehy.2013.05.030","article-title":"Does dysregulated complement activation contribute to haemolytic uraemic syndrome secondary to Streptococcus pneumoniae?","volume":"81","author":"Gilbert","year":"2013","journal-title":"Med Hypotheses"},{"key":"2021021623390719700_sfaa004-B2","doi-asserted-by":"crossref","first-page":"2237","DOI":"10.1093\/ndt\/gft198","article-title":"The role of complement in Streptococcus pneumoniae-associated haemolytic uraemic syndrome","volume":"28","author":"Szil\u00e1gyi","year":"2013","journal-title":"Nephrol Dial Transplant"},{"key":"2021021623390719700_sfaa004-B3","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1053\/j.ajkd.2012.02.329","article-title":"Acute presentation and persistent glomerulonephritis following streptococcal infection in a patient with heterozygous complement factor H\u2013related protein 5 deficiency","volume":"60","author":"Vernon","year":"2012","journal-title":"Am J Kidney Dis"},{"key":"2021021623390719700_sfaa004-B4","doi-asserted-by":"crossref","first-page":"4963","DOI":"10.4049\/jimmunol.1403121","article-title":"Factor H\u2013related protein 5 interacts with pentraxin 3 and the extracellular matrix and modulates complement 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