{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2023,10,2]],"date-time":"2023-10-02T08:11:48Z","timestamp":1696234308964},"reference-count":16,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2016,2,25]],"date-time":"2016-02-25T00:00:00Z","timestamp":1456358400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Transplantation"],"published-print":{"date-parts":[[2016,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>An eight\u2010yr\u2010old female with a history of multifocal lymphangioendotheliomatosis and thrombocytopenia presented for <jats:styled-content style=\"fixed-case\">MVT<\/jats:styled-content>. The patient had multiple vascular lesions in the skin and stomach in infancy. Although her cutaneous lesions resolved with vincristine and methylprednisolone, her gastric lesions persisted. Eight yr later, she was diagnosed with portal hypertension and decompensating liver function despite therapy with bevacizumab, propranolol, furosemide, and spironolactone. Upon presentation, she was found to have a <jats:styled-content style=\"fixed-case\">K<\/jats:styled-content>asabach\u2013<jats:styled-content style=\"fixed-case\">M<\/jats:styled-content>erritt\u2010like coagulopathy in association with multiple lesions in her <jats:styled-content style=\"fixed-case\">GI<\/jats:styled-content> tract and persistent gastric lesions. Although treatment with methylprednisolone and sirolimus normalized her coagulation factors and <jats:sc>d<\/jats:sc>\u2010dimer levels, she never developed sustained improvement in her thrombocytopenia. Her liver function continued to deteriorate and she developed hepatorenal syndrome. Given better outcomes after <jats:styled-content style=\"fixed-case\">OLT<\/jats:styled-content> in comparison with <jats:styled-content style=\"fixed-case\">MVT<\/jats:styled-content>, she underwent <jats:styled-content style=\"fixed-case\">OLT<\/jats:styled-content>, with the plan to manage her <jats:styled-content style=\"fixed-case\">GI<\/jats:styled-content> lesions with <jats:styled-content style=\"fixed-case\">APC<\/jats:styled-content> post\u2010transplant. Post\u2010transplant, her liver function and coagulopathy normalized, and <jats:styled-content style=\"fixed-case\">GI<\/jats:styled-content> tract lesions disappeared upon screening with capsule endoscopy. The patient is doing well, without recurrence of either <jats:styled-content style=\"fixed-case\">GI<\/jats:styled-content> lesions or thrombocytopenia, at 18 months after transplantation.<\/jats:p>","DOI":"10.1111\/petr.12696","type":"journal-article","created":{"date-parts":[[2016,2,26]],"date-time":"2016-02-26T08:56:46Z","timestamp":1456477006000},"page":"456-459","source":"Crossref","is-referenced-by-count":3,"title":["Orthotopic liver transplant for multifocal lymphangioendotheliomatosis with thrombocytopenia"],"prefix":"10.1111","volume":"20","author":[{"given":"Christine H.","family":"Yang","sequence":"first","affiliation":[{"name":"Division of Pediatrics Children's Hospital of Los Angeles University of Southern California Los Angeles CA USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shengmei","family":"Zhou","sequence":"additional","affiliation":[{"name":"Division of Pathology Children's Hospital of Los Angeles University of Southern California Los Angeles CA USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sophoclis","family":"Alexopoulos","sequence":"additional","affiliation":[{"name":"Division of Hepatobiliary Surgery and Abdominal Organ Transplantation Keck School of Medicine University of Southern California Los Angeles CA USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Larry","family":"Wang","sequence":"additional","affiliation":[{"name":"Division of Pathology Children's Hospital of Los Angeles University of Southern California Los Angeles CA USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Howard I.","family":"Baron","sequence":"additional","affiliation":[{"name":"Pediatric Gastroenterology and Nutrition Associates Sunrise Children's Hospital Las Vegas NV USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yuri","family":"Genyk","sequence":"additional","affiliation":[{"name":"Division of Hepatobiliary Surgery and Abdominal Organ Transplantation Keck School of Medicine University of Southern California Los Angeles CA USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nanda","family":"Kerkar","sequence":"additional","affiliation":[{"name":"Division of Gastroenterology, Hepatology and Nutrition Children's Hospital of Los Angeles University of Southern California Los Angeles CA USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2016,2,25]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.21766"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2005-1936"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPG.0b013e31822aa2db"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1001\/archderm.140.5.599"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2011.08.020"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10024-005-1124-2"},{"key":"e_1_2_6_8_1","unstructured":"PazdurR.FDA approval for bevacizumab[Online] 2014. 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