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Article

Thrombotic thrombocytopenic purpura related to severe ADAMTS13 deficiency in children.

Pediatric nephrology (Berlin, Germany) - 1 Jan 2009

Loirat Chantal, Girma Jean-Pierre, Desconclois Céline, Coppo Paul, Veyradier Agnès

Abstract excerpt

Thrombotic thrombocytopenic purpura (TTP) related to a severely deficient activity of the von Willebrand factor cleaving protease, ADAMTS (A Disintegrin And Metalloprotease with ThromboSpondin type 1 repeats) 13, is a life-threatening event, the onset of which may occur as early as childhood. TTP is either inherited (Upshaw-Schulman syndrome) via ADAMTS13 gene mutations (neonatal onset) or acquired via...

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Source

Identifiers

PMID: 18574602
DOI: 10.1007/s00467-008-0863-5

Genes

ADAMTS13

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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