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It can be classified as either typical, primarily caused by Shiga toxin-producing <jats:italic>Escherichia coli<\/jats:italic> (STEC) infection, or as atypical HUS (aHUS), which results from uncontrolled complement activation.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Methods<\/jats:title>\n                <jats:p>We report the case of a 9-year-old boy with aHUS due to compound heterozygous complement factor H-related genes (CFHR) <jats:italic>1\/3<\/jats:italic> and <jats:italic>CFHR1<\/jats:italic>\u2013<jats:italic>CFHR4<\/jats:italic> deletions, leading to the development of anti-complement factor H (CFH) autoantibodies. The patient presented nephrological and neurological thrombotic microangiopathy with STEC positivity. Additionally, we provide an extensive literature review of aHUS cases initially classified as typical.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Results<\/jats:title>\n                <jats:p>A total of 11 patients were included, 73% of whom were pediatric. Kidney replacement therapy was required in 73% of patients. The recurrence rate was 55%. All cases were found positive for pathological variants of the complement system genes. The most commonly implicated gene was <jats:italic>CFH<\/jats:italic>, while the <jats:italic>CFHR<\/jats:italic> genes were involved in 36% of cases, although none exhibited anti-CFH autoantibodies. Anti-complement therapy was administered in 54% of cases, and none of the patients who received it early progressed to kidney failure.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusions<\/jats:title>\n                <jats:p>STEC infection does not exclude aHUS diagnosis, and early use of anti-complement therapy might be reasonable in life-threatening conditions. Genetic testing can be helpful in patients with atypical presentations and can confirm the necessity of prolonged anti-complement therapy.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Graphical Abstract<\/jats:title>\n                \n              <\/jats:sec>","DOI":"10.1007\/s00467-024-06480-9","type":"journal-article","created":{"date-parts":[[2024,9,30]],"date-time":"2024-09-30T10:02:55Z","timestamp":1727690575000},"page":"449-461","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Shiga toxin-producing Escherichia coli infection as a precipitating factor for atypical hemolytic-uremic syndrome"],"prefix":"10.1007","volume":"40","author":[{"ORCID":"https:\/\/orcid.org\/0009-0002-7113-6258","authenticated-orcid":false,"given":"Gabriele","family":"Mortari","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0007-8563-8351","authenticated-orcid":false,"given":"Carolina","family":"Bigatti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giulia Proietti","family":"Gaffi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Barbara","family":"Lionetti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6121-5326","authenticated-orcid":false,"given":"Andrea","family":"Angeletti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simona","family":"Matarese","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5178-1949","authenticated-orcid":false,"given":"Enrico Eugenio","family":"Verrina","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6700-3001","authenticated-orcid":false,"given":"Gianluca","family":"Caridi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4189-8561","authenticated-orcid":false,"given":"Francesca","family":"Lugani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5107-1584","authenticated-orcid":false,"given":"Valerio Gaetano","family":"Vellone","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6877-9511","authenticated-orcid":false,"given":"Decimo Silvio","family":"Chiarenza","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2368-1139","authenticated-orcid":false,"given":"Edoardo","family":"La Porta","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2024,9,30]]},"reference":[{"key":"6480_CR1","doi-asserted-by":"publisher","first-page":"324","DOI":"10.1186\/s12882-017-0727-y","volume":"18","author":"T Sakari Jokiranta","year":"2017","unstructured":"Sakari Jokiranta T, Viklicky O, Al Shorafa S et al (2017) Differential diagnosis of thrombotic microangiopathy in nephrology. 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