[Thrombotic microangiopathies].
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.
Nielsen
Ove Juul
OJ
Klinisk Biokemisk Afdeling KB3011, Rigshospitalet, DK-2100 København Ø, Denmark.
Friis-Hansen
Lennart
L
D003937:Diagnosis, Differential; D042241:Early Diagnosis; D006463:Hemolytic-Uremic Syndrome / Q000175:diagnosis / Q000401:mortality / Q000628:therapy; D006801:Humans; D011379:Prognosis; D011696:Purpura, Thrombocytopenic / Q000175:diagnosis / Q000401:mortality / Q000628:therapy; D011697:Purpura, Thrombotic Thrombocytopenic / Q000175:diagnosis / Q000401:mortality / Q000628:therapy; D057049:Thrombotic Microangiopathies / Q000175:diagnosis / Q000401:mortality / Q000628:therapy