{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,26]],"date-time":"2026-09-26T12:04:47Z","timestamp":1790424287636,"version":"4.1.0"},"reference-count":9,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2013,8,12]],"date-time":"2013-08-12T00:00:00Z","timestamp":1376265600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100001700","name":"Ministry of Education, Culture, Sports, Science and Technology of Japan","doi-asserted-by":"crossref","award":["23591425"],"award-info":[{"award-number":["23591425"]}],"id":[{"id":"10.13039\/501100001700","id-type":"DOI","asserted-by":"crossref"}]},{"name":"Ministry of Health, Labor, and Welfare of Japan","award":["H23-Nanchi-Ippan-002"],"award-info":[{"award-number":["H23-Nanchi-Ippan-002"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Obstet and Gynaecol"],"published-print":{"date-parts":[[2014,1]]},"abstract":"Abstract<\/jats:title>Upshaw\u2013S<\/jats:styled-content>chulman syndrome (USS<\/jats:styled-content>) involves a congenital deficiency of von W<\/jats:styled-content>illebrand factor\u2010cleaving metalloprotease (ADAMTS13<\/jats:styled-content>) activity due to gene mutations. Female patients develop overt thrombotic thrombocytopenic purpura (TTP<\/jats:styled-content>) caused by a decline of ADAMTS13<\/jats:styled-content> activity in pregnancy. A 23\u2010year\u2010old nulliparous J<\/jats:styled-content>apanese woman died due to severe, rapid progression of TTP<\/jats:styled-content> with intrauterine fetal death at 20 weeks of gestation after its onset, even though she underwent intensive treatment which included plasma exchange. She had a history of idiopathic thrombocytopenic purpura at the age of 3 years. The patient's ADAMTS13<\/jats:styled-content> activity was of very low level. It should be borne in mind that there is the possibility of rapidly progressive fulminant USS<\/jats:styled-content> during pregnancy.<\/jats:p>","DOI":"10.1111\/jog.12125","type":"journal-article","created":{"date-parts":[[2013,8,13]],"date-time":"2013-08-13T04:40:22Z","timestamp":1376368822000},"page":"247-249","source":"Crossref","is-referenced-by-count":13,"title":["Case of maternal and fetal deaths due to severe congenital thrombotic thrombocytopenic purpura (U<\/scp>pshaw\u2013S<\/scp>chulman syndrome) during pregnancy"],"prefix":"10.1111","volume":"40","author":[{"given":"Hirokazu","family":"Tanaka","sequence":"first","affiliation":[{"name":"Department of Perinatology and Gynecology Kagawa University School of Medicine Miki Kagawa Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chiaki","family":"Tenkumo","sequence":"additional","affiliation":[{"name":"Department of Perinatology and Gynecology Kagawa University School of Medicine Miki Kagawa Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nobuhiro","family":"Mori","sequence":"additional","affiliation":[{"name":"Department of Perinatology and Gynecology Kagawa University School of Medicine Miki Kagawa Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Koichi","family":"Kokame","sequence":"additional","affiliation":[{"name":"Department of Molecular Pathogenesis National Cerebral and Cardiovascular Center Osaka Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoshihiro","family":"Fujimura","sequence":"additional","affiliation":[{"name":"Department of Blood Transfusion Medicine Nara Medical University Nara Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Toshiyuki","family":"Hata","sequence":"additional","affiliation":[{"name":"Department of Perinatology and Gynecology Kagawa University School of Medicine Miki Kagawa Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2013,8,12]]},"reference":[{"key":"e_1_2_5_2_1","first-page":"1758","article-title":"Willebrand factor\u2013cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic\u2013uremic syndrome","volume":"339","author":"Furlan M","journal-title":"N Engl J Med"},{"key":"e_1_2_5_3_1","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1007\/BF02982558","article-title":"Upshaw\u2013Schulman syndrome revisited: A concept of congenital thrombotic thrombocytopenic purpura","volume":"74","author":"Kinoshita S","journal-title":"Int J Hematol"},{"key":"e_1_2_5_4_1","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 GG","journal-title":"Nature"},{"key":"e_1_2_5_5_1","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\u2010cleaving protease activity","volume":"99","author":"Kokame K","journal-title":"Proc Nati Acad Sci U S A"},{"key":"e_1_2_5_6_1","doi-asserted-by":"crossref","first-page":"742","DOI":"10.1111\/j.1365-2141.2008.07515.x","article-title":"Pregnancy\u2010induced thrombocytopenia and TTP, and the risk of fetal death, in Upshaw\u2010Schulman syndrome: A series of 15 pregnancies in 9 genotyped patients","volume":"144","author":"Fujimura Y","journal-title":"Br J Haematol"},{"key":"e_1_2_5_7_1","first-page":"1320","article-title":"Von Willebrand factor\u2010cleaving protease (ADAMTS13) activity in normal non\u2010pregnant women, pregnant and post\u2010delivery women","volume":"92","author":"Sanchez\u2010Luceros A","journal-title":"Thromb Haemost"},{"key":"e_1_2_5_8_1","first-page":"176","article-title":"Haemostais in normal pregnancy","volume":"52","author":"Stirling Y","journal-title":"Thromb Haemost"},{"key":"e_1_2_5_9_1","doi-asserted-by":"crossref","first-page":"211","DOI":"10.1111\/j.1365-3148.2010.01067.x","article-title":"Successful management of a planned pregnancy in severe congenital thrombotic thrombocytopaenic purpura: The Upshaw\u2010Schulman syndrome","volume":"21","author":"Richter J","journal-title":"Transfus Med"},{"key":"e_1_2_5_10_1","doi-asserted-by":"crossref","first-page":"519","DOI":"10.1111\/j.1479-828X.2010.01222.x","article-title":"Clinical study on five cases of thrombotic thrombocytopenic purpura complicating pregnancy","volume":"50","author":"He Y","journal-title":"Aust N Z J Obstet Gynaecol"}],"container-title":["Journal of Obstetrics and Gynaecology Research"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fjog.12125","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fjog.12125","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/obgyn.onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/jog.12125","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,2]],"date-time":"2023-10-02T17:54:20Z","timestamp":1696269260000},"score":1,"resource":{"primary":{"URL":"https:\/\/obgyn.onlinelibrary.wiley.com\/doi\/10.1111\/jog.12125"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2013,8,12]]},"references-count":9,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2014,1]]}},"alternative-id":["10.1111\/jog.12125"],"URL":"https:\/\/doi.org\/10.1111\/jog.12125","archive":["Portico"],"relation":{},"ISSN":["1341-8076","1447-0756"],"issn-type":[{"value":"1341-8076","type":"print"},{"value":"1447-0756","type":"electronic"}],"subject":[],"published":{"date-parts":[[2013,8,12]]}}}