{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,13]],"date-time":"2026-02-13T08:11:19Z","timestamp":1770970279770,"version":"3.50.1"},"reference-count":21,"publisher":"American Society of Hematology","issue":"21","content-domain":{"domain":["ashpublications.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2019,11,12]]},"abstract":"<jats:title>Key Points<\/jats:title>\n               <jats:p>One third of patients with USS have a neonatal episode of severe hemolytic jaundice with thrombocytopenia induced by an unknown trigger. A USS patient with persisting PDA and recurrent neonatal hemolysis\/thrombocytopenia suggests blood flow through the PDA as trigger.<\/jats:p>","DOI":"10.1182\/bloodadvances.2019000601","type":"journal-article","created":{"date-parts":[[2019,10,28]],"date-time":"2019-10-28T11:36:57Z","timestamp":1572262617000},"page":"3191-3195","update-policy":"https:\/\/doi.org\/10.1182\/blood.2019cm0000","source":"Crossref","is-referenced-by-count":26,"title":["Patent ductus arteriosus generates neonatal hemolytic jaundice with thrombocytopenia in Upshaw-Schulman syndrome"],"prefix":"10.1182","volume":"3","author":[{"given":"Yoshihiro","family":"Fujimura","sequence":"first","affiliation":[{"name":"Japanese Red Cross Kinki Block Blood Center, Ibaraki, Japan;"},{"name":"Department of Blood Transfusion Medicine, Nara Medical University, Kashihara, Japan;"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bernhard","family":"L\u00e4mmle","sequence":"additional","affiliation":[{"name":"Department of Hematology and Central Hematology Laboratory, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland;"},{"name":"Center for Thrombosis and Hemostasis, University Medical Center Mainz, Mainz, Germany;"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Saori","family":"Tanabe","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Nihonkai General Hospital, Sakata, Japan;"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kazuya","family":"Sakai","sequence":"additional","affiliation":[{"name":"Department of Blood Transfusion Medicine, Nara Medical University, Kashihara, Japan;"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Toshiyuki","family":"Kimura","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Nihonkai General Hospital, Sakata, Japan;"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Koichi","family":"Kokame","sequence":"additional","affiliation":[{"name":"Department of Molecular Pathogenesis and"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Toshiyuki","family":"Miyata","sequence":"additional","affiliation":[{"name":"Department of Molecular Pathogenesis and"},{"name":"Department of Cerebrovascular Medicine, National Cerebral and Cardiovascular Center, Suita, Japan; and"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yukihiro","family":"Takahashi","sequence":"additional","affiliation":[{"name":"Japanese Red Cross Kinki Block Blood Center, Ibaraki, Japan;"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shigeki","family":"Taniguchi","sequence":"additional","affiliation":[{"name":"Department of Thoracic and Cardiovascular Surgery, Nara Medical University, Kashihara, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7243-3126","authenticated-orcid":false,"given":"Masanori","family":"Matsumoto","sequence":"additional","affiliation":[{"name":"Department of Blood Transfusion Medicine, Nara Medical University, Kashihara, Japan;"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"234","published-online":{"date-parts":[[2019,10,28]]},"reference":[{"key":"2019111521180546600_B1","doi-asserted-by":"crossref","first-page":"943","DOI":"10.1182\/blood.V16.1.943.943","article-title":"Studies on thrombopoiesis. I. A factor in normal human plasma required for platelet production; chronic thrombocytopenia due to its deficiency","volume":"16","author":"Schulman","year":"1960","journal-title":"Blood"},{"issue":"24","key":"2019111521180546600_B2","doi-asserted-by":"crossref","first-page":"1350","DOI":"10.1056\/NEJM197806152982407","article-title":"Congenital deficiency of a factor in normal plasma that reverses microangiopathic hemolysis and thrombocytopenia","volume":"298","author":"Upshaw","year":"1978","journal-title":"N Engl J Med"},{"issue":"1","key":"2019111521180546600_B3","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1007\/BF02982558","article-title":"Upshaw-Schulman syndrome revisited: a concept of congenital thrombotic thrombocytopenic purpura","volume":"74","author":"Kinoshita","year":"2001","journal-title":"Int J Hematol"},{"issue":"2","key":"2019111521180546600_B4","doi-asserted-by":"crossref","first-page":"437","DOI":"10.1053\/beha.2001.0142","article-title":"Aetiology and pathogenesis of thrombotic thrombocytopenic purpura and haemolytic uraemic syndrome: the role of von Willebrand factor-cleaving protease","volume":"14","author":"Furlan","year":"2001","journal-title":"Best Pract Res Clin Haematol"},{"issue":"6855","key":"2019111521180546600_B5","doi-asserted-by":"crossref","first-page":"488","DOI":"10.1038\/35097008","article-title":"Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura","volume":"413","author":"Levy","year":"2001","journal-title":"Nature"},{"issue":"85","key":"2019111521180546600_B6","first-page":"79","article-title":"Atypical congenital haemolytic anaemia","volume":"22","author":"Dacie","year":"1953","journal-title":"Q J Med"},{"issue":"1","key":"2019111521180546600_B7","doi-asserted-by":"crossref","first-page":"118","DOI":"10.1016\/S0022-3476(67)80241-5","article-title":"Thrombotic thrombocytopenic purpura in a neonatal infant","volume":"71","author":"Monnens","year":"1967","journal-title":"J Pediatr"},{"issue":"suppl 1","key":"2019111521180546600_B8","doi-asserted-by":"crossref","first-page":"283","DOI":"10.1111\/j.1538-7836.2011.04341.x","article-title":"Natural history of Upshaw-Schulman syndrome based on ADAMTS13 gene analysis in Japan","volume":"9","author":"Fujimura","year":"2011","journal-title":"J Thromb Haemost"},{"issue":"11","key":"2019111521180546600_B9","doi-asserted-by":"crossref","first-page":"1902","DOI":"10.1055\/s-0038-1673686","article-title":"ADAMTS13 gene mutations influence ADAMTS13 conformation and disease age-onset in the French cohort of Upshaw-Schulman syndrome","volume":"118","author":"Joly","year":"2018","journal-title":"Thromb Haemost"},{"issue":"15","key":"2019111521180546600_B10","doi-asserted-by":"crossref","first-page":"1644","DOI":"10.1182\/blood-2018-11-884700","article-title":"Characterization and treatment of congenital thrombotic thrombocytopenic purpura","volume":"133","author":"Alwan","year":"2019","journal-title":"Blood"},{"issue":"10","key":"2019111521180546600_B11","doi-asserted-by":"crossref","first-page":"2107","DOI":"10.3324\/haematol.2019.216796","article-title":"The International Hereditary Thrombotic Thrombocytopenic Purpura Registry: key findings at enrollment until 2017","volume":"104","author":"van Dorland","year":"2019","journal-title":"Haematologica"},{"issue":"8","key":"2019111521180546600_B12","doi-asserted-by":"crossref","first-page":"1444","DOI":"10.1111\/j.1537-2995.2006.00914.x","article-title":"Novel monoclonal antibody-based enzyme immunoassay for determining plasma levels of ADAMTS13 activity","volume":"46","author":"Kato","year":"2006","journal-title":"Transfusion"},{"issue":"18","key":"2019111521180546600_B13","doi-asserted-by":"crossref","first-page":"11902","DOI":"10.1073\/pnas.172277399","article-title":"Mutations and common polymorphisms in ADAMTS13 gene responsible for von Willebrand factor-cleaving protease activity","volume":"99","author":"Kokame","year":"2002","journal-title":"Proc Natl Acad Sci U S A"},{"issue":"6","key":"2019111521180546600_B14","doi-asserted-by":"crossref","first-page":"789","DOI":"10.1007\/s12185-012-1221-8","article-title":"Two newborn-onset patients of Upshaw-Schulman syndrome with distinct subsequent clinical courses","volume":"96","author":"Tanabe","year":"2012","journal-title":"Int J Hematol"},{"key":"2019111521180546600_B15","doi-asserted-by":"crossref","first-page":"50","DOI":"10.1016\/j.jpeds.2018.04.051","article-title":"Cardiopulmonary adaptation during first day of life in human neonates","volume":"200","author":"Jain","year":"2018","journal-title":"J Pediatr"},{"issue":"1","key":"2019111521180546600_B16","doi-asserted-by":"crossref","first-page":"75","DOI":"10.1038\/nm.2060","article-title":"Platelets contribute to postnatal occlusion of the ductus arteriosus","volume":"16","author":"Echtler","year":"2010","journal-title":"Nat Med"},{"issue":"4","key":"2019111521180546600_B17","doi-asserted-by":"crossref","DOI":"10.1542\/peds.2016-1627","article-title":"Preterm thrombocytopenia and delay of ductus arteriosus closure","volume":"138","author":"Kulkarni","year":"2016","journal-title":"Pediatrics"},{"issue":"9449","key":"2019111521180546600_B18","doi-asserted-by":"crossref","first-page":"1945","DOI":"10.1016\/S0140-6736(04)17477-1","article-title":"Prophylactic ibuprofen in premature infants: a multicentre, randomised, double-blind, placebo-controlled trial","volume":"364","author":"Van Overmeire","year":"2004","journal-title":"Lancet"},{"issue":"5","key":"2019111521180546600_B19","doi-asserted-by":"crossref","first-page":"407","DOI":"10.1016\/S0049-3848(01)00261-4","article-title":"Acquired von Willebrand disease in an infant. Resolution by interventional occlusion of patent ductus arteriosus","volume":"102","author":"Rauch","year":"2001","journal-title":"Thromb Res"},{"issue":"11","key":"2019111521180546600_B20","doi-asserted-by":"crossref","first-page":"2150","DOI":"10.1111\/jth.14208","article-title":"Acquired von Willebrand syndrome in congenital heart disease surgery: results from an observational case-series","volume":"16","author":"Icheva","year":"2018","journal-title":"J Thromb Haemost"},{"issue":"20","key":"2019111521180546600_B21","doi-asserted-by":"crossref","first-page":"1954","DOI":"10.1056\/NEJMcibr1205363","article-title":"From clinical observation to mechanism\u2013Heyde\u2019s syndrome","volume":"367","author":"Loscalzo","year":"2012","journal-title":"N Engl J Med"}],"container-title":["Blood Advances"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/ashpublications.org\/bloodadvances\/article-pdf\/3\/21\/3191\/1505836\/advancesadv2019000601.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"http:\/\/ashpublications.org\/bloodadvances\/article-pdf\/3\/21\/3191\/1505836\/advancesadv2019000601.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2019,11,15]],"date-time":"2019-11-15T23:47:31Z","timestamp":1573861651000},"score":1,"resource":{"primary":{"URL":"https:\/\/ashpublications.org\/bloodadvances\/article\/3\/21\/3191\/422642\/Patent-ductus-arteriosus-generates-neonatal"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,10,28]]},"references-count":21,"journal-issue":{"issue":"21","published-print":{"date-parts":[[2019,11,12]]}},"URL":"https:\/\/doi.org\/10.1182\/bloodadvances.2019000601","relation":{},"ISSN":["2473-9529","2473-9537"],"issn-type":[{"value":"2473-9529","type":"print"},{"value":"2473-9537","type":"electronic"}],"subject":[],"published":{"date-parts":[[2019,10,28]]}}}