{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,5,14]],"date-time":"2025-05-14T03:34:29Z","timestamp":1747193669391,"version":"3.40.5"},"reference-count":14,"publisher":"S. Karger AG","issue":"11","license":[{"start":{"date-parts":[[2023,1,1]],"date-time":"2023-01-01T00:00:00Z","timestamp":1672531200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/karger.com\/pages\/terms-and-conditions"},{"start":{"date-parts":[[2023,1,1]],"date-time":"2023-01-01T00:00:00Z","timestamp":1672531200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/karger.com\/pages\/terms-and-conditions"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Nephron"],"published-print":{"date-parts":[[2023]]},"abstract":"<jats:p>Atypical hemolytic uremic syndrome (aHUS) is a condition characterized by acute kidney injury (AKI), thrombocytopenia, and microangiopathic hemolytic anemia secondary to complement pathway dysregulation. Several triggers have been identified as causing aHUS in genetically susceptible patients; however, hypereosinophilia syndrome (HES)-triggered aHUS has not been reported. In this article, we present a case of aHUS presented with generalized urticarial rashes and angioedema. The initial investigations revealed hypereosinophilia (maximal absolute eosinophil count of 6,840 cells\/\u00b5L) with normal bone-marrow analyses; hence, idiopathic HES was diagnosed. During hospitalization, the patient developed convulsion, stuporous, and full-blown thrombotic microangiopathy (TMA), with AKI requiring temporary hemodialysis. A kidney biopsy confirmed the existence of renal TMA. Next-generation sequencing of the coding regions of aHUS-related genes was performed, revealing an underlying complement factor I (CFI) deficiency, a heterozygous variant p.P64L of &lt;i&gt;CFI&lt;\/i&gt; gene. The patient was successfully treated with high-dose steroids and extended duration of plasmapheresis. <\/jats:p>","DOI":"10.1159\/000531879","type":"journal-article","created":{"date-parts":[[2023,10,11]],"date-time":"2023-10-11T21:05:10Z","timestamp":1697058310000},"page":"701-706","source":"Crossref","is-referenced-by-count":0,"title":["Complement Factor I Gene Variant in an Atypical Hemolytic Uremic Syndrome Triggered by Hypereosinophilia Syndrome"],"prefix":"10.1159","volume":"147","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0732-4051","authenticated-orcid":false,"given":"Athiphat","family":"Banjongjit","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Wonngarm","family":"Kittanamongkolchai","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2996-8934","authenticated-orcid":false,"given":"Talerngsak","family":"Kanjanabuch","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"127","published-online":{"date-parts":[[2023,8,23]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","DOI":"10.1016\/j.kint.2016.10.005"},{"key":"ref2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1523-1755.2005.00278.x"},{"key":"ref3","doi-asserted-by":"publisher","DOI":"10.1177\/1076029609348647"},{"key":"ref4","doi-asserted-by":"publisher","DOI":"10.1002\/humu.21256"},{"key":"ref5","doi-asserted-by":"publisher","DOI":"10.1038\/gim.2015.30"},{"key":"ref6","doi-asserted-by":"publisher","DOI":"10.1016\/j.semnephrol.2013.08.003"},{"key":"ref7","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2019.01150"},{"key":"ref8","doi-asserted-by":"publisher","DOI":"10.1007\/s11255-010-9754-3"},{"key":"ref9","doi-asserted-by":"publisher","DOI":"10.1136\/bcr-2017-222560"},{"key":"ref10","doi-asserted-by":"publisher","DOI":"10.1007\/s13730-017-0251-8"},{"key":"ref11","doi-asserted-by":"publisher","DOI":"10.2169\/internalmedicine.48.2282"},{"key":"ref12","doi-asserted-by":"publisher","DOI":"10.1186\/s13256-019-2187-4"},{"key":"ref13","doi-asserted-by":"publisher","DOI":"10.1016\/j.kint.2021.05.021"},{"key":"ref14","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.24880"}],"container-title":["Nephron"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/karger.com\/nef\/article-pdf\/147\/11\/701\/4043099\/000531879.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/karger.com\/nef\/article-pdf\/147\/11\/701\/4043099\/000531879.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,4,23]],"date-time":"2025-04-23T15:40:08Z","timestamp":1745422808000},"score":1,"resource":{"primary":{"URL":"https:\/\/karger.com\/article\/doi\/10.1159\/000531879"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023]]},"references-count":14,"journal-issue":{"issue":"11","published-online":{"date-parts":[[2023,11,1]]},"published-print":{"date-parts":[[2023,11,7]]}},"URL":"https:\/\/doi.org\/10.1159\/000531879","archive":["Portico"],"relation":{},"ISSN":["1660-8151","2235-3186"],"issn-type":[{"type":"print","value":"1660-8151"},{"type":"electronic","value":"2235-3186"}],"subject":[],"published":{"date-parts":[[2023]]}}}