{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,9,25]],"date-time":"2025-09-25T16:28:47Z","timestamp":1758817727121,"version":"3.40.5"},"reference-count":12,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2019,2,3]],"date-time":"2019-02-03T00:00:00Z","timestamp":1549152000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"Alexion Pharma Middle East FZ LLC"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J Paediatrics Child Health"],"published-print":{"date-parts":[[2019,10]]},"abstract":"<jats:sec><jats:title>Aim<\/jats:title><jats:p>Atypical haemolytic uraemic syndrome (aHUS) is a debilitating condition that can cause significant morbidity and mortality in children if not adequately and promptly treated. This report shares real\u2010world data on the use of eculizumab in children with aHUS.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We report our experience with the use of eculizumab in 14 children with aHUS.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The median age at aHUS diagnosis was 12\u2009months (range: 2\u2013108\u2009months), with six (42.9%) patients presenting in infancy and six (42.9%) being males. Eculizumab therapy was associated with haematological and thrombotic microangiopathy responses in 14 (100%) and 13 (92.9%) patients after a median of 9\u2009days (range: 7\u201312\u2009days) and 9.5\u2009days (range: 7\u201314\u2009days), respectively. None of the six patients who were previously treated with plasma therapy required any further infusions. Of the six patients who previously required dialysis, only one patient continued to do so and eventually received a renal transplant. The median time to \u226525% decrease in serum creatinine level in the remaining patients was 16\u2009days (range: 14\u201321\u2009days), and estimated glomerular filtration rate increased from a median of 17\u2013101\u2009mL\/min\/1.73\u2009m<jats:sup>2<\/jats:sup>. The safety profile was similar to that reported in the literature, and 10 patients continue to receive therapy, with 3 being on the drug for 4 or more years.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Our study adds to the growing body of evidence highlighting the efficacy and safety of eculizumab for the management of children with aHUS.<\/jats:p><\/jats:sec>","DOI":"10.1111\/jpc.14390","type":"journal-article","created":{"date-parts":[[2019,2,4]],"date-time":"2019-02-04T01:18:18Z","timestamp":1549243098000},"page":"1237-1240","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Eculizumab in paediatric atypical haemolytic uraemic syndrome: Lessons learned from a single\u2010centre experience in the United Arab Emirates"],"prefix":"10.1111","volume":"55","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5457-1827","authenticated-orcid":false,"given":"Gurinder","family":"Kumar","sequence":"first","affiliation":[{"name":"Division of Pediatric Nephrology Sheikh Khalifa Medical City  Abu Dhabi United Arab Emirates"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Omar","family":"Al\u2010Masri","sequence":"additional","affiliation":[{"name":"Division of Pediatric Nephrology Sheikh Khalifa Medical City  Abu Dhabi United Arab Emirates"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zubaida","family":"Alismaili","sequence":"additional","affiliation":[{"name":"Division of Pediatric Nephrology Sheikh Khalifa Medical City  Abu Dhabi United Arab Emirates"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Eslam","family":"Tawfik","sequence":"additional","affiliation":[{"name":"Division of Pediatric Nephrology Sheikh Khalifa Medical City  Abu Dhabi United Arab Emirates"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mohammed K","family":"Al\u2010Ghabra","sequence":"additional","affiliation":[{"name":"Division of Pediatric Nephrology Sheikh Khalifa Medical City  Abu Dhabi United Arab Emirates"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sadia H","family":"Ilyas","sequence":"additional","affiliation":[{"name":"Division of Pediatric Nephrology Sheikh Khalifa Medical City  Abu Dhabi United Arab Emirates"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Eihab","family":"Al\u2010Khasawneh","sequence":"additional","affiliation":[{"name":"Division of Pediatric Nephrology Sheikh Khalifa Medical City  Abu Dhabi United Arab 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