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Article

Severe Hemolysis and Pulmonary Hypertension in a Neonate With Upshaw-Schulman Syndrome.

Pediatrics - 1 Dec 2016

Tsujii Nobuyuki, Shiraishi Isao, Kokame Koichi, Shima Midori, Fujimura Yoshihiro, Takahashi Yukihiro, Matsumoto Masanori

Abstract excerpt

Pulmonary involvement is extremely rare in thrombotic thrombocytopenic purpura. In this report, we present a girl patient with congenital thrombotic thrombocytopenic purpura, known as Upshaw-Schulman syndrome (USS), complicated with severe hemolysis and pulmonary hypertension (PH). The assay results of a disintegrin-like and metalloprotease with thrombospondin type 1 motifs 13 (ADAMTS13) activity measured by...

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Identifiers

PMID: 27940692
DOI: 10.1542/peds.2016-1565

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ADAMTS13

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Severe Hemolysis and Pulmonary Hypertension in a Neonate With Upshaw-Schulman Syndrome.PMID 27940692Read ↗

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

Pregnancy-induced thrombocytopenia and TTP, and the risk of fetal death, in Upshaw-Schulman syndrome: a series of 15 pregnancies in 9 genotyped patients.Semantic proximity Read ↗

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

Thrombotic thrombocytopenic purpura related to severe ADAMTS13 deficiency in children.Semantic proximity Read ↗

Congenital thrombotic thrombocytopenic purpura caused by new compound heterozygous mutations of the ADAMTS13 gene.Semantic proximity Read ↗

Natural history of Upshaw-Schulman syndrome based on ADAMTS13 gene analysis in Japan.Semantic proximity Read ↗

Hereditary Thrombotic Thrombocytopenic Purpura.Semantic proximity Read ↗

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An ADAMTS13 mutation that causes hereditary thrombotic thrombocytopenic purpura: a case report and literature review.Semantic proximity Read ↗

Mutation Spectrum of ADAMTS13 Gene in Patients with Upshaw-Schulman Syndrome (USS) in Russia.Semantic proximity Read ↗

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