Thrombotic thrombocytopenic purpura in a newborn

We report the case of a newborn presenting with hemolytic anemia, thrombocytopenia, hyperbilirubinemia, and renal failure in the first hours of life. An early plasmatherapy was undertaken, with good outcome. The specific von Willebrand factor-cleaving protease activity (ADAMTS 13 for a disintegrin a...

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Published inArchives de pédiatrie : organe officiel de la Société française de pédiatrie Vol. 23; no. 1; pp. 78 - 81
Main Authors Ghariani, I, Jmili-Braham, N, Azzebi, O, Kortas, M, Veyradier, A, Bakir, L
Format Journal Article
LanguageFrench
Published France 01.01.2016
Subjects
Anemia, Hemolytic - etiology
Humans
Hyperbilirubinemia - etiology
Infant, Newborn
Male
Purpura, Thrombotic Thrombocytopenic - complications
Purpura, Thrombotic Thrombocytopenic - diagnosis
Renal Insufficiency - etiology
Thrombocytopenia - etiology
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Summary:We report the case of a newborn presenting with hemolytic anemia, thrombocytopenia, hyperbilirubinemia, and renal failure in the first hours of life. An early plasmatherapy was undertaken, with good outcome. The specific von Willebrand factor-cleaving protease activity (ADAMTS 13 for a disintegrin and metalloprotease with thrombospondin type 1 repeats) was found to be low. This is the specific biologic diagnostic element of congenital thrombotic thrombocytopenic purpura (TTP). This disease of constitutional thrombotic microangiopathy is rare. The prognosis, usually life-threatening, was completely transformed given the better understanding of the pathogenesis of the disease and therapeutic progress.
Bibliography:ObjectType-Case Study-2
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ISSN:1769-664X
DOI:10.1016/j.arcped.2015.09.032