{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,25]],"date-time":"2026-08-25T19:27:45Z","timestamp":1787686065559,"version":"build-2784847793"},"reference-count":19,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2021,3,17]],"date-time":"2021-03-17T00:00:00Z","timestamp":1615939200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2021,3,17]],"date-time":"2021-03-17T00:00:00Z","timestamp":1615939200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Samara Jan Turkel Center"},{"name":"McCance Family Foundation"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Pediatr Rheumatol"],"published-print":{"date-parts":[[2021,12]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Despite timely administration of IVIG, some patients with Kawasaki disease (KD) develop rapidly progressive or giant coronary artery aneurysms (CAA).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Case presentation<\/jats:title>\n                    <jats:p>\n                      We describe our experience using cyclophosphamide (CYC) for the treatment of such cases as well as a review of the literature on the use of CYC in KD. Through a retrospective chart review of our KD population, we identified ten children treated for KD with intravenous CYC (10\u2009mg\/kg\/dose) for one or two doses. Seven patients were male, the median age was 2.0\u2009years (range 4\u2009months \u2212\u20095\u2009years). All patients received initial IVIG between day 4\u201310 of illness. Other anti-inflammatory treatments administered before CYC included second IVIG (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20099), corticosteroids (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u200910), infliximab (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20094), cyclosporine (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20092), and anakinra (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20091). Median illness day at administration of the first CYC dose was 22.5\u2009days (range:10\u201336\u2009days). The primary indication for treatment with CYC for all patients was large or giant CAA and\/or rapid progression of CAA. Three patients received a second dose of CYC (10\u2009mg\/kg) for progressively enlarging CAA. CAA did not progress after final CYC treatment\n                      <jats:bold>.<\/jats:bold>\n                      One patient with a history of neutropenia in infancy developed severe neutropenia 9\u2009days after treatment with CYC, which recovered without intervention or complications. No patient developed infections or other serious toxicity from CYC.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>In KD patients with severe and progressive enlargement of CAA despite anti-inflammatory therapy, CYC seemed to arrest further dilation and was well-tolerated. Future multicenter studies are needed to confirm our findings in this subgroup of KD patients.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1186\/s12969-021-00526-0","type":"journal-article","created":{"date-parts":[[2021,3,17]],"date-time":"2021-03-17T05:06:25Z","timestamp":1615957585000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":22,"title":["Cyclophosphamide use in treatment of refractory Kawasaki disease with coronary artery aneurysms"],"prefix":"10.1186","volume":"19","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3117-173X","authenticated-orcid":false,"given":"Olha","family":"Halyabar","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kevin G.","family":"Friedman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert P.","family":"Sundel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annette L.","family":"Baker","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Margaret H.","family":"Chang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patrick W.","family":"Gould","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jane W.","family":"Newburger","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mary Beth F.","family":"Son","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2021,3,17]]},"reference":[{"issue":"17","key":"526_CR1","doi-asserted-by":"publisher","first-page":"e927","DOI":"10.1161\/CIR.0000000000000484","volume":"135","author":"BW McCrindle","year":"2017","unstructured":"McCrindle BW, Rowley AH, Newburger JW, et al. Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association. Circulation. 2017;135(17):e927\u2013e99. https:\/\/doi.org\/10.1161\/CIR.0000000000000484 [published Online First: 2017\/03\/31].","journal-title":"Circulation"},{"key":"526_CR2","doi-asserted-by":"publisher","unstructured":"Son MBF, Gauvreau K, Kim S, et al. Predicting Coronary Artery Aneurysms in Kawasaki Disease at a North American Center: An Assessment of Baseline z Scores. J Am Heart Assoc. 2017;6(6). https:\/\/doi.org\/10.1161\/JAHA.116.005378 [published Online First: 2017\/06\/02].","DOI":"10.1161\/JAHA.116.005378"},{"issue":"23","key":"526_CR3","doi-asserted-by":"publisher","first-page":"1633","DOI":"10.1056\/NEJM199106063242305","volume":"324","author":"JW Newburger","year":"1991","unstructured":"Newburger JW, Takahashi M, Beiser AS, et al. 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The authors have no financial relationships relevant to this article to disclose.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"31"}}