{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,13]],"date-time":"2026-06-13T14:22:39Z","timestamp":1781360559063,"version":"3.54.1"},"reference-count":7,"publisher":"BMJ","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Reports"],"accepted":{"date-parts":[[2018,1,25]]},"abstract":"<jats:p>A 17-year-old girl presented with fever, myalgia, vomiting for 1\u2009month and oliguria and dyspnoea for 4 days. She was tachycardic,hypertensive, with pedal oedema and decreased breath sounds. She had high serum creatinine (3\u2009mg\/dL), anaemia, thrombocytopenia, leucocytosis and eosinophilia with schistocytes. Lactate dehydrogenase, transaminases were high , with low haptoglobin and high ferritin (5269\u2009ng\/mL). Complement C3\/C4 and fibrinogen were normal. Urinalysis showed large blood and protein and stool studies were negative. Her ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) was normal. Kidney biopsy showed acute interstitial nephritis (AIN) in addition to thrombotic angiopathy. The differentials - haemolytic uraemic syndrome (HUS), thrombotic thrombocytopenia (TTP) and haemophagocytic lymphohistiocytosis (HLH) were ruled out. Her genetic testing was abnormal for large CFHR1\u2013CFHR3 homozygous deletion and heterozygous missense variant in exon 2 of DGKE making the diagnosis of atypical HUS. She received eculizumab and was discharged on oral steroids for AIN and biweekly eculizumab infusions with excellent recovery.<\/jats:p>","DOI":"10.1136\/bcr-2017-222560","type":"journal-article","created":{"date-parts":[[2018,2,11]],"date-time":"2018-02-11T23:45:13Z","timestamp":1518392713000},"page":"bcr-2017-222560","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":6,"title":["Atypical presentation of atypical haemolytic uraemic syndrome"],"prefix":"10.1136","volume":"2018","author":[{"given":"Ratna","family":"Basak","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Stony Brook University Hospital, Stony Brook, New York, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiaotong","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Pathology, Stony Brook University Hospital, Stony Brook, New York, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Caitlin","family":"Keane","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Stony Brook University Hospital, Stony Brook, New York, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert","family":"Woroniecki","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Stony Brook University Hospital, Stony Brook, New York, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2018,2,11]]},"reference":[{"key":"2025082802214884000_2018.feb09_1.bcr-2017-222560.1","doi-asserted-by":"publisher","DOI":"10.1007\/s00467-015-3076-8"},{"key":"2025082802214884000_2018.feb09_1.bcr-2017-222560.2","doi-asserted-by":"publisher","DOI":"10.1136\/archdischild-2015-309471"},{"key":"2025082802214884000_2018.feb09_1.bcr-2017-222560.3","doi-asserted-by":"publisher","DOI":"10.1016\/j.kint.2015.11.026"},{"key":"2025082802214884000_2018.feb09_1.bcr-2017-222560.4","doi-asserted-by":"publisher","DOI":"10.1681\/ASN.2012090903"},{"key":"2025082802214884000_2018.feb09_1.bcr-2017-222560.5","doi-asserted-by":"publisher","DOI":"10.1681\/ASN.2017010031"},{"key":"2025082802214884000_2018.feb09_1.bcr-2017-222560.6","doi-asserted-by":"publisher","DOI":"10.2215\/CJN.08520814"},{"key":"2025082802214884000_2018.feb09_1.bcr-2017-222560.7","doi-asserted-by":"crossref","first-page":"563","DOI":"10.1038\/nrneph.2016.99","article-title":"Anti-complement-factor H-associated glomerulopathies","volume":"12","author":"Durey","year":"2016","journal-title":"Nat Rev Nephrol"}],"container-title":["BMJ Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/bcr-2017-222560","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,8,28]],"date-time":"2025-08-28T09:21:56Z","timestamp":1756372916000},"score":1,"resource":{"primary":{"URL":"https:\/\/casereports.bmj.com\/lookup\/doi\/10.1136\/bcr-2017-222560"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,2,11]]},"references-count":7,"alternative-id":["10.1136\/bcr-2017-222560"],"URL":"https:\/\/doi.org\/10.1136\/bcr-2017-222560","relation":{},"ISSN":["1757-790X"],"issn-type":[{"value":"1757-790X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,2,11]]}}}