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Case of maternal and fetal deaths due to severe congenital thrombotic thrombocytopenic purpura (Upshaw-Schulman syndrome) during pregnancy.

The journal of obstetrics and gynaecology research - 1 Jan 2014

Tanaka Hirokazu, Tenkumo Chiaki, Mori Nobuhiro, Kokame Koichi, Fujimura Yoshihiro, Hata Toshiyuki

Abstract excerpt

Upshaw-Schulman syndrome (USS) involves a congenital deficiency of von Willebrand factor-cleaving metalloprotease (ADAMTS13) activity due to gene mutations. Female patients develop overt thrombotic thrombocytopenic purpura (TTP) caused by a decline of ADAMTS13 activity in pregnancy. A 23-year-old nulliparous Japanese woman died due to severe, rapid progression of TTP with intrauterine fetal death at 20 weeks of...

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Identifiers

PMID: 23937165
DOI: 10.1111/jog.12125

Genes

ADAMTS13

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Case of maternal and fetal deaths due to severe congenital thrombotic thrombocytopenic purpura (Upshaw-Schulman syndrome) during pregnancy.PMID 23937165Read ↗

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Unexpected frequency of Upshaw-Schulman syndrome in pregnancy-onset thrombotic thrombocytopenic purpura.Semantic proximity Read ↗

Inherited ADAMTS13 deficiency (Upshaw-Schulman syndrome): a short review.Semantic proximity Read ↗

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Novel ADAMTS13 mutations in an obstetric patient with Upshaw-Schulman syndrome.Semantic proximity Read ↗

Congenital thrombotic thrombocytopenic purpura (Upshaw-Schulman syndrome) caused by novel ADAMTS13 mutations.Semantic proximity Read ↗

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