[Thrombotic microangiopathies].

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.

• Authors: Ove Juul Nielsen, Lennart Friis-Hansen
• Journal: Ugeskrift for laeger (Ugeskr Laeger) Vol. 171 Issue 49 Pg. 3614-20 (Nov 30 2009) ISSN: 1603-6824 [Electronic] Denmark
• Vernacular Title: De trombotiske mikroangiopatier.
• PMID: 19954703 (Publication Type: English Abstract, Journal Article, Review)

Topics

• Diagnosis, Differential
• Early Diagnosis
• Hemolytic-Uremic Syndrome (diagnosis, mortality, therapy)
• Humans
• Prognosis
• Purpura, Thrombocytopenic (diagnosis, mortality, therapy)
• Purpura, Thrombotic Thrombocytopenic (diagnosis, mortality, therapy)
• Thrombotic Microangiopathies (diagnosis, mortality, therapy)