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ADAMTS13 Secretion and Residual Activity among Patients with Congenital Thrombotic Thrombocytopenic Purpura with and without Renal Impairment
Author(s) -
Erica Rurali,
Federica Banterla,
Roberta Donadelli,
Elena Bresin,
Miriam Galbusera,
Sara Gastoldi,
Flora Peyvandi,
Mary Underwood,
Giuseppe Remuzzi,
Marioris
Publication year - 2015
Publication title -
clinical journal of the american society of nephrology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.755
H-Index - 151
eISSN - 1555-905X
pISSN - 1555-9041
DOI - 10.2215/cjn.01700215
Subject(s) - medicine , adamts13 , thrombotic thrombocytopenic purpura , odds ratio , gastroenterology , von willebrand factor , confidence interval , platelet , endocrinology
Acute renal impairment is observed in 11%-23% of patients with congenital thrombotic thrombocytopenic purpura (TTP) and deficiency of a disintegrin and metalloprotease with thrombospondin motifs 13 (ADAMTS13, a metalloprotease that cleaves von Willebrand factor [VWF] multimers), a substantial percentage of whom develop CKD during follow-up.

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