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  • Title: [Thrombotic microangiopathies].
    Author: Nielsen OJ, Friis-Hansen L.
    Journal: Ugeskr Laeger; 2009 Nov 30; 171(49):3614-20. PubMed ID: 19954703.
    Abstract:
    The thrombotic microangiopathic diseases, which include acquired and congenital TTP and HUS, are most frequently acute disease entities. Untreated, these diseases are associated with a lethal course in many cases. Deficiency of the von Willebrand cleaving enzyme, ADAMTS13, is a decisive pathophysiological defect in most cases of thrombotic microangiopathic diseases. Early recognition and a secure distinction from other conditions that resemble TMA diseases is essential in order to institute adequate treatment and to obtain a favourable outcome in terms of both survival and development of serious sequelae.
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