{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T00:32:41Z","timestamp":1791246761520,"version":"4.1.0"},"reference-count":26,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2024,11,27]],"date-time":"2024-11-27T00:00:00Z","timestamp":1732665600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Clinical Transplantation"],"published-print":{"date-parts":[[2024,12]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Hepatic veno\u2010occlusive disease (VOD) is a life\u2010threatening complication of hematopoietic cell transplantation (HCT) and is categorized as a transplant\u2010related, systemic endothelial disease. Severe VOD can lead to multi\u2010organ dysfunction (MOF) and is associated with a high mortality rate.<\/jats:p><\/jats:sec><jats:sec><jats:title>Objective<\/jats:title><jats:p>To evaluate the incidence of VOD in children after HCT and analyze the outcomes and risk factors associated with increased mortality.<\/jats:p><\/jats:sec><jats:sec><jats:title>Study Design<\/jats:title><jats:p>A retrospective cohort study of 1243 children with malignant and non\u2010malignant diseases who underwent HCT at two large pediatric centers over 20 years.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>One hundred one patients (8%) developed VOD post HCT. Most patients developed VOD post allogeneic HCT (76%) versus autologous (24%). The incidence of VOD was twice as high in children with malignant diseases compared to non\u2010malignant (68%\u00a0vs. 32%). A much higher incidence of VOD occurred in patients after a busulfan\u2010based regimen versus total body irradiation\u2010based and treosulfan\u2010based, 73%, 18%, and 1%, respectively. The 100\u2010day survival rate of HCT patients with VOD was 69%. The overall survival rate of the entire group was 50%, showing improvement over the span of the study years, from 40% between 2000 and 2009 to 63% between 2010 and 2021 (<jats:italic>p<\/jats:italic> = 0.022). Factors associated with increased mortality included infections before transplant (<jats:italic>p<\/jats:italic> = 0.013), conditioning regimen (<jats:italic>p<\/jats:italic> = 0.01), abnormal liver function (<jats:italic>p<\/jats:italic> = 0.019), presence of ascites (<jats:italic>p<\/jats:italic> = 0.008), MOF (<jats:italic>p<\/jats:italic> &lt; 0.001), and the need for admission to a pediatric intensive care unit (<jats:italic>p<\/jats:italic> &lt; 0.001). There was no significant difference in survival rates between children treated with defibrotide alone or with those treated with defibrotide and steroids (61% and 65%, respectively; <jats:italic>p<\/jats:italic> = 0.685).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Severe VOD in pediatric patients following HCT remains a life\u2010threatening complication with a high mortality rate. Early diagnosis and treatment with defibrotide are critical for managing this condition. In our cohort, the addition of steroids to defibrotide was not associated with improved outcomes.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ctr.70037","type":"journal-article","created":{"date-parts":[[2024,11,27]],"date-time":"2024-11-27T11:07:02Z","timestamp":1732705622000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Prognostic Factors Associated With Increased Mortality in Pediatric Veno\u2010Occlusive Disease Following Hematopoietic Cell Transplantation"],"prefix":"10.1111","volume":"38","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7549-0935","authenticated-orcid":false,"given":"Irina","family":"Zaidman","sequence":"first","affiliation":[{"name":"Faculty of Medicine Hebrew University of Jerusalem  Jerusalem Israel"},{"name":"Department of Bone Marrow Transplantation and Cancer Immunotherapy Hadassah\u2010Hebrew University Medical Center  Jerusalem 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