NDT Advance Access originally published online on May 5, 2004
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Nephrol Dial Transplant (2004) 19: 1687-1693
Nephrol Dial Transplant Vol. 19 No. 7 © ERA-EDTA 2004; all rights reserved
Editorial Review
Not known from ADAM(TS-13)novel insights into the pathophysiology of thrombotic microangiopathies
Department of Medicine, Division of Nephrology, Rheumatology and Osteology, University of Hamburg, Hamburg, Germany
Correspondence and offprint requests to: Gunter Wolf, MD, University of Hamburg, University Hospital Eppendorf, Department of Medicine, Division of Nephrology, Rheumatology and Osteology, Pavilion N26, Martinistraße 52, D-20246 Hamburg, Germany. Email: Wolf@uke.uni-hamburg.de
Keywords: ADAMTS-13; haemolyticuraemic syndrome; thrombotic microangiopathy; thrombotic thrombocytopenic purpura
| The first 150 words of the full text of this article appear below. |
Introduction
Thrombotic microangiopathies encompass various disease entities that are characterized by haemolytic anaemia caused by fragmentation of erythrocytes and thrombocytopenia due to increased platelet aggregation and thrombus formation, eventually leading to disturbed microcirculation with reduced organ perfusion (Figure 1). Thrombotic thrombocytopenic purpura (TTP) and the haemolyticuraemic syndrome (HUS) are thrombotic microangiopathies characterized by a distinct pattern of symptoms [1,2]. TTP, first described by Moschcowitz in 1924 [3], has been defined as a disease of adults characterized by severe thrombocytopenia leading to purpura, microangiopathic haemolytic anaemia with fragmented erythrocytes (so-called schistocytes), increased serum lactate dehydrogenase that is largely derived from ischaemic organs rather than from lysed erythrocytes, various neurologic abnormalities, fever and mild renal dysfunction [4]. In contrast, HUS has been considered as a syndrome attacking preferentially children and young persons, with severe acute renal failure and marked anaemia as the predominant
TTP
HUS
Is low ADAMTS-13 activity a valid criterion to distinguish between TTP and HUS?
Conclusions