{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,21]],"date-time":"2026-08-21T12:05:55Z","timestamp":1787313955993,"version":"build-2736575974"},"reference-count":19,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2025,2,25]],"date-time":"2025-02-25T00:00:00Z","timestamp":1740441600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Nephrology"],"published-print":{"date-parts":[[2025,3]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    Atypical hemolytic uremic syndrome is a thrombotic microangiopathy caused by the abnormal activation of the alternative complement pathway. Mutations in complement\u2010related genes and autoantibodies against complement regulators are involved in the pathogenesis of this condition; the frequency of, and prognosis of patients harbouring, each genetic mutation varies based on the region and race. Complement factor I (\n                    <jats:italic>CFI<\/jats:italic>\n                    ) mutations have been observed in 4%\u20138% of cases in Europe; however, they have not yet been reported in Japan. We present the first Japanese case of atypical hemolytic uremic syndrome in a patient harbouring a\n                    <jats:italic>CFI<\/jats:italic>\n                    mutation. An 83\u2010year\u2010old female patient presented with severe acute kidney injury, thrombocytopenia, and hemolytic anaemia following a femoral neck fracture. Plasma exchange and haemodialysis were initiated, resulting in improved kidney function and platelet count. However, the platelet count decreased when plasma exchange was discontinued. Therefore, we administered ravulizumab, an anti\u2010complement 5 monoclonal antibody, which led to the maintenance of stable kidney function and platelet count. Genetic analysis revealed a\n                    <jats:italic>CFI<\/jats:italic>\n                    mutation, and the patient was treated with ravulizumab for 2\u2009years without relapse. Individuals diagnosed with atypical hemolytic uremic syndrome harbouring\n                    <jats:italic>CFI<\/jats:italic>\n                    mutations experience poor outcomes, including low rates of remission, high rates of mortality, and progression to end\u2010stage kidney disease. Our case serves as a crucial example demonstrating how prompt identification and appropriate management can lead to better patient outcomes.\n                  <\/jats:p>","DOI":"10.1111\/nep.70010","type":"journal-article","created":{"date-parts":[[2025,2,26]],"date-time":"2025-02-26T01:01:11Z","timestamp":1740531671000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["A Case of Atypical Hemolytic Uremic Syndrome With a Complement Factor I Mutation Triggered by a Femoral Neck Fracture"],"prefix":"10.1111","volume":"30","author":[{"ORCID":"https:\/\/orcid.org\/0009-0006-3041-0416","authenticated-orcid":false,"given":"Toshiki","family":"Kano","sequence":"first","affiliation":[{"name":"Department of Nephrology Juntendo University Nerima Hospital  Tokyo Japan"},{"name":"Department of Nephrology Juntendo University Faculty of Medicine  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9446-2390","authenticated-orcid":false,"given":"Hiroaki","family":"Io","sequence":"additional","affiliation":[{"name":"Department of Nephrology Juntendo University Nerima Hospital  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yu","family":"Sasaki","sequence":"additional","affiliation":[{"name":"Department of Nephrology Juntendo University Nerima Hospital  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masahiro","family":"Muto","sequence":"additional","affiliation":[{"name":"Department of Nephrology Juntendo University Nerima Hospital  Tokyo Japan"},{"name":"Department of Nephrology Juntendo University Faculty of Medicine  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sayaka","family":"Muto","sequence":"additional","affiliation":[{"name":"Department of Nephrology Juntendo University Nerima 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