{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,12]],"date-time":"2026-10-12T01:20:29Z","timestamp":1791768029893,"version":"4.3.4"},"reference-count":27,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2006,6,19]],"date-time":"2006-06-19T00:00:00Z","timestamp":1150675200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Blood &amp; Cancer"],"published-print":{"date-parts":[[2007,6,15]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>This retrospective report describes experience with defibrotide in children with hepatic veno\u2010occlusive disease (HVOD) following hematopoietic progenitor cell transplant (HPCT) in a single institution.<\/jats:p><\/jats:sec><jats:sec><jats:title>Procedure<\/jats:title><jats:p>Children who had undergone HPCT between February 1999 and June 2001 and between July 2003 and September 2004 and who received defibrotide during their admission were identified. Demographic data and information regarding the clinical course of these patients were abstracted from their health records.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Fourteen children (mean age: 9.3 years; range: 0.4\u201318.1) who underwent HPCT during the study period received defibrotide for the treatment of HVOD; nine were girls. Most patients underwent HPCT for hematologic malignancies (8\/14) and received matched unrelated donor transplants (8\/14). Conditioning regimens included cyclophosphamide with total body irradiation (5\/14) and busulfan followed by cyclophosphamide (7\/14). HVOD was diagnosed on transplant day \u22124 to +33 (median: +10.5); defibrotide was started on transplant day \u22124 to +40 (median: +12). The median initial defibrotide dose was 33 mg\/kg\/day (11\u201340 mg\/kg\/day); the median maximum defibrotide dose was 38.5 mg\/kg\/day (11\u201381 mg\/kg\/day). The median duration of defibrotide therapy was 16 days (4\u201337 days). Defibrotide was discontinued due to clinical improvement (9), death (3), drug unavailability (1), and neurological toxicity (1). Gastrointestinal hemorrhage was observed in two patients and intra\u2010cranial hemorrhage was observed in one patient during defibrotide therapy. The survival rate to day +100 was 79%.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Defibrotide appears to be an effective and relatively safe treatment for children with HVOD. Pediatr Blood Cancer 2007;48:700\u2013704. \u00a9 2006 Wiley\u2010Liss, Inc.<\/jats:p><\/jats:sec>","DOI":"10.1002\/pbc.20934","type":"journal-article","created":{"date-parts":[[2006,6,19]],"date-time":"2006-06-19T22:04:08Z","timestamp":1150754648000},"page":"700-704","source":"Crossref","is-referenced-by-count":48,"title":["Defibrotide for the treatment of hepatic veno\u2010occlusive disease in children"],"prefix":"10.1002","volume":"48","author":[{"given":"Sean R.","family":"Bulley","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brigitte","family":"Strahm","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John","family":"Doyle","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L. 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