{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2024,9,16]],"date-time":"2024-09-16T14:33:54Z","timestamp":1726497234693},"reference-count":29,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2006,7,11]],"date-time":"2006-07-11T00:00:00Z","timestamp":1152576000000},"content-version":"vor","delay-in-days":4393,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[1994,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>The pathogenesis of veno\u2010occlusive disease (VOD) of the liver appears to be secondary to endothelial damage of terminal hepatic venules, which leads to activation of the coagulation cascade, fibrin deposition, and eventual fibrous obliteration of the hepatic venules. Patients with VOD usually present with jaundice, hepatomegaly, weight gain, and ascites. This complication is usually associated with a high mortality rate.<\/jats:p><jats:p>We report here the frequency and treatment of VOD in our autologous bone marrow transplant (BMT) patient population. Three of 15 (20%) children (median age 9 years) developed VOD and were treated with recombinant tissue plasminogen activator (rt\u2010PA). Two of these three patients were prepared for BMT with busulfan (16 mg\/kg) and cyclophosphamide (Cytoxan, 200 mg\/kg), while the other child received cytosine arabinoside (ARA\u2010C 18 g\/m<jats:sup>2<\/jats:sup>), Cytoxan (3,600 mg\/m<jats:sup>2<\/jats:sup>) and total body irradiation (TBI, 1,400 r). VOD developed between days 7\u201324 posttransplant. Clotting studies obtained pretransplant and during VOD included prothrombin time (PT), partial thromboplastin time (PTT), fibrinogen, fibrin\u2010degradation product (FDP), proteins C and S, and platelet count. There was no correlation between the incidence of VOD and coagulation status. All patients had normal pretransplant clotting studies. However, protein C levels were noted to be consistently low for those patients at the time of VOD.<\/jats:p><jats:p>All three patients received rt\u2010PA at a dose of 0.25\u20130.5 mg\/kg for 4 days. This dose produced increased levels of FDP but did not significantly prolong PT nor PTT. Two of the patients had dramatic responses and had complete resolution of VOD within 6\u201312 days from the start of therapy. The other patient died of fulminant hepatic failure. It seems that rt\u2010PA is effective in VOD of the liver, which may be associated with low protein C level. \u00a9 1994 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/ajh.2830460307","type":"journal-article","created":{"date-parts":[[2007,2,21]],"date-time":"2007-02-21T13:42:56Z","timestamp":1172065376000},"page":"194-198","source":"Crossref","is-referenced-by-count":23,"title":["Recombinant tissue plasminogen activator (rt\u2010PA) for veno\u2010occlusive liver disease in pediatric autologous bone marrow transplant patients"],"prefix":"10.1002","volume":"46","author":[{"given":"Lolie C.","family":"Yu","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Indrani","family":"Malkani","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Oswaldo","family":"Regueira","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"David L.","family":"Ode","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Raj P.","family":"Warrier","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2006,7,11]]},"reference":[{"key":"e_1_2_1_2_2","doi-asserted-by":"publisher","DOI":"10.1136\/adc.32.165.369"},{"key":"e_1_2_1_3_2","first-page":"584","article-title":"Pathogenesis of veno\u2010occlusive liver disease after radiation","volume":"104","author":"Fajardo LF","year":"1980","journal-title":"Arch Pathol Lab Med"},{"key":"e_1_2_1_4_2","doi-asserted-by":"publisher","DOI":"10.1002\/1097-0142(19800515)45:10<2670::AID-CNCR2820451031>3.0.CO;2-L"},{"key":"e_1_2_1_5_2","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-96-1-58"},{"key":"e_1_2_1_6_2","doi-asserted-by":"crossref","first-page":"1178","DOI":"10.1016\/0016-5085(80)90911-7","article-title":"An analysis of hepatic veno\u2010occlusive disease and centrilobular hepatic degeneration following bone marrow transplantation","volume":"79","author":"Shulman HM","year":"1980","journal-title":"Gastroenterology"},{"key":"e_1_2_1_7_2","first-page":"279","article-title":"Hepatic veins of monkeys with veno\u2010occlusive disease. 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