{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,13]],"date-time":"2025-11-13T07:09:06Z","timestamp":1763017746267,"version":"3.37.3"},"reference-count":23,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2018,6,10]],"date-time":"2018-06-10T00:00:00Z","timestamp":1528588800000},"content-version":"vor","delay-in-days":9,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Nephrology"],"published-print":{"date-parts":[[2018,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Aim<\/jats:title><jats:p>Recently eculizumab, a monoclonal antibody to C5, was found to improve the disease course of atypical haemolytic uraemic syndrome (aHUS) and has been recommended as the first line treatment by an international consensus guideline. However, several practical issues in the use of eculizumab for the acute phase of aHUS have yet to be resolved.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Children who received eculizumab with diagnosis of aHUS between March 2010 and December 2015 at Tokyo Metropolitan Children's Medical Center were enrolled. aHUS was diagnosed according to the haemolytic uraemic syndrome (HUS) criteria after excluding Shiga toxin\u2010inducing <jats:styled-content style=\"fixed-case\"><jats:italic>Escherichia coli<\/jats:italic><\/jats:styled-content> (STEC) \u2010associated HUS and thrombocytopaenic purpura. We retrieved and analyzed data from the electronic medical records at our institution.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>We reviewed four patients with suspected aHUS. Eculizumab was discontinued in one patient in whom STEC\u2010HUS was later diagnosed. Treatment was continued in the remaining three patients without recurrence. Practical issues included difficulty in diagnosing aHUS, particularly in the acute phase, risk of infection by encapsulated organisms, especially <jats:italic>Neisseria meningitis<\/jats:italic>, and infusion reaction. In addition to issues relating to the acute phase, discontinuing eculizumab in stable patients in the chronic phase must be considered.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Eculizumab, the first line treatment for children with aHUS, is usually effective. However, certain problems associated with its use require caution to be exercised. As clinical information on eculizumab are still very limited, and the rationale for its long\u2010term use has yet to be established, physicians are advised to exercise care when using eculizumab to manage aHUS.<\/jats:p><\/jats:sec>","DOI":"10.1111\/nep.13054","type":"journal-article","created":{"date-parts":[[2017,4,7]],"date-time":"2017-04-07T14:03:05Z","timestamp":1491573785000},"page":"539-545","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Practical issues in using eculizumab for children with atypical haemolytic uraemic syndrome in the acute phase: A review of four patients"],"prefix":"10.1111","volume":"23","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5033-9270","authenticated-orcid":false,"given":"Chikako","family":"Terano","sequence":"first","affiliation":[{"name":"Department of Nephrology Tokyo Metropolitan Children's Medical Center  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