{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,7]],"date-time":"2026-07-07T20:39:10Z","timestamp":1783456750085,"version":"3.55.0"},"reference-count":27,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2020,8,31]],"date-time":"2020-08-31T00:00:00Z","timestamp":1598832000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Blood &amp; Cancer"],"published-print":{"date-parts":[[2020,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Hepatic sinusoidal obstruction syndrome (SOS) is a serious complication of autologous stem cell transplant (ASCT) in children with historically high mortality rates. Defibrotide has shown proven benefit in its treatment and may have a modest role in prevention. We report our experience with SOS in children undergoing autologous transplant.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Case records of 82 consecutive patients undergoing ASCT following high\u2010dose chemotherapy between 2010 and 2017 were reviewed. Defibrotide was used for treatment of all with SOS and prophylactically in patients receiving busulfan\u2010based conditioning until 2014.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Fourteen of the 82 children (17%) were diagnosed with SOS. The incidence was higher in those receiving busulfan\u2010based conditioning (13\/42\u00a0vs 1\/40, <jats:italic>P<\/jats:italic>\u00a0=\u00a00.008). Mean (\u00b1SD) time to diagnosis of SOS was 19 (\u00b15.6) days following stem cell rescue. Bilirubin levels and ultrasound were normal in 7\/14 and 3\/14 patients. Coagulopathy was noted in 10\/14; one child developed multiorgan involvement. Nine children had mild SOS, whereas two and three had moderate and severe SOS, respectively. Intensive care was required for four of five non\u2010mild cases. Patients with SOS had significantly delayed platelet recovery, higher transfusion requirement, and longer hospital stay. Unavailability of defibrotide prophylaxis for 17\/42 receiving busulfan did not change the incidence of SOS (7\/25 with defibrotide vs 6 \/17 without defibrotide, <jats:italic>P<\/jats:italic>\u00a0=\u00a00.74). There was no significant difference in the severity of SOS between these groups.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Hepatic SOS was more commonly seen in children receiving busulfan\u2010based conditioning. Stopping the use of prophylactic defibrotide did not increase incidence or severity of SOS. Overall outcome was excellent with supportive care and timely treatment with defibrotide.<\/jats:p><\/jats:sec>","DOI":"10.1002\/pbc.28677","type":"journal-article","created":{"date-parts":[[2020,8,31]],"date-time":"2020-08-31T15:41:23Z","timestamp":1598888483000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Defibrotide treatment but not prophylaxis is useful in hepatic sinusoidal obstruction syndrome in children undergoing autologous stem cell transplant following high\u2010dose chemotherapy: A single\u2010center experience from the Royal Marsden Hospital, UK"],"prefix":"10.1002","volume":"67","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5650-3115","authenticated-orcid":false,"given":"Nirmalya","family":"Roy Moulik","sequence":"first","affiliation":[{"name":"Children and Young People's Unit The Royal Marsden Hospital  Sutton United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Indranee","family":"Johnson","sequence":"additional","affiliation":[{"name":"Children and Young People's Unit The Royal Marsden Hospital  Sutton United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lucia","family":"Van Bruggen","sequence":"additional","affiliation":[{"name":"Department of Pharmacy The Royal Marsden Hospital  Sutton United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Toni","family":"Petterson","sequence":"additional","affiliation":[{"name":"Children and Young People's Unit The Royal Marsden Hospital  Sutton United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julie","family":"Mycroft","sequence":"additional","affiliation":[{"name":"Department of Pharmacy The Royal Marsden Hospital  Sutton United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2980-9316","authenticated-orcid":false,"given":"Sucheta J.","family":"Vaidya","sequence":"additional","affiliation":[{"name":"Children and Young People's Unit The Royal Marsden Hospital  Sutton United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2020,8,31]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jceh.2014.10.002"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbmt.2015.09.024"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbmt.2011.06.006"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1080\/17474124.2017.1370372"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.14727"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.bmt.1705696"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.21425"},{"issue":"5","key":"e_1_2_7_9_1","first-page":"CD009311","article-title":"Interventions for prophylaxis of hepatic veno\u2010occlusive disease in people undergoing haematopoietic stem cell transplantation","author":"Cheuk DK","year":"2015","journal-title":"Cochrane Database Syst Rev"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(11)61938-7"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1038\/bmt.2016.130"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1038\/bmt.2017.161"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.bmt.1703393"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1038\/bmt.2008.69"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1038\/bmt.2016.45"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbmt.2016.07.013"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1186\/1756-8722-3-36"},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbmt.2014.09.026"},{"key":"e_1_2_7_19_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V124.21.5849.5849"},{"key":"e_1_2_7_20_1","doi-asserted-by":"publisher","DOI":"10.2214\/ajr.164.6.7754881"},{"key":"e_1_2_7_21_1","doi-asserted-by":"publisher","DOI":"10.7863\/jum.1997.16.9.575"},{"key":"e_1_2_7_22_1","doi-asserted-by":"publisher","DOI":"10.2214\/ajr.150.2.289"},{"key":"e_1_2_7_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbmt.2018.09.027"},{"key":"e_1_2_7_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbmt.2017.12.253"},{"key":"e_1_2_7_25_1","unstructured":"Clinical Commissioning Policy: Use of DF in severe venoocclusive disease following stem cell transplant\u2010 NHS England Clinical Reference Group for Blood and Marrow Transplantation. 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