Thrombotic thrombocytopenic purpura related to severe ADAMTS13 deficiency in children

@article{Loirat2009ThromboticTP,
  title={Thrombotic thrombocytopenic purpura related to severe ADAMTS13 deficiency in children},
  author={C. Loirat and J. Girma and C. Desconclois and P. Coppo and A. Veyradier},
  journal={Pediatric Nephrology},
  year={2009},
  volume={24},
  pages={19-29}
}
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 anti-ADAMTS13 autoantibodies (childhood onset). TTP is due to platelet- and von-Willebrand… Expand
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Observational Clinical Trial
Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy defined by the spontaneous formation of platelet thrombi in the microvessels. These platelet microthrombi… Expand
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