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A classification of hemolytic uremic syndrome and thrombotic thrombocytopenic purpura and related disorders

Besbas, N. ; Karpman, Diana LU orcid ; Landau, D. ; Loirat, C. ; Proesmans, W. ; Remuzzi, G. ; Rizzoni, G. ; Taylor, C. M. ; Van de Kar, N. and Zimmerhackl, L. B. (2006) In Kidney International 70(3). p.423-431
Abstract
The diagnostic terms hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP) are based on historical and overlapping clinical descriptions. Advances in understanding some of the causes of the syndrome now permit many patients to be classified according to etiology. The increased precision of a diagnosis based on causation is important for considering logical approaches to treatment and prognosis. It is also essential for research. We propose a classification that accommodates both a current understanding of causation (level 1) and clinical association in cases for whom cause of disease is unclear (level 2). We tested the classification in a pediatric disease registry of HUS. The revised classification is a stimulus to... (More)
The diagnostic terms hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP) are based on historical and overlapping clinical descriptions. Advances in understanding some of the causes of the syndrome now permit many patients to be classified according to etiology. The increased precision of a diagnosis based on causation is important for considering logical approaches to treatment and prognosis. It is also essential for research. We propose a classification that accommodates both a current understanding of causation (level 1) and clinical association in cases for whom cause of disease is unclear (level 2). We tested the classification in a pediatric disease registry of HUS. The revised classification is a stimulus to comprehensive investigation of all cases of HUS and TTP and is expected to increase the proportion of cases in whom a level 1 etiological diagnosis is confirmed. (Less)
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author
; ; ; ; ; ; ; ; and
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
hemolytic uremic syndrome, thrombotic thrombocytopenic purpura, etiology
in
Kidney International
volume
70
issue
3
pages
423 - 431
publisher
Nature Publishing Group
external identifiers
  • wos:000239390200006
  • scopus:33746508999
ISSN
1523-1755
DOI
10.1038/sj.ki.5001581
language
English
LU publication?
yes
id
d45379bd-2484-4b5b-b50b-edf949bcba25 (old id 399059)
date added to LUP
2016-04-01 16:34:54
date last changed
2020-10-20 02:46:59
@article{d45379bd-2484-4b5b-b50b-edf949bcba25,
  abstract     = {The diagnostic terms hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP) are based on historical and overlapping clinical descriptions. Advances in understanding some of the causes of the syndrome now permit many patients to be classified according to etiology. The increased precision of a diagnosis based on causation is important for considering logical approaches to treatment and prognosis. It is also essential for research. We propose a classification that accommodates both a current understanding of causation (level 1) and clinical association in cases for whom cause of disease is unclear (level 2). We tested the classification in a pediatric disease registry of HUS. The revised classification is a stimulus to comprehensive investigation of all cases of HUS and TTP and is expected to increase the proportion of cases in whom a level 1 etiological diagnosis is confirmed.},
  author       = {Besbas, N. and Karpman, Diana and Landau, D. and Loirat, C. and Proesmans, W. and Remuzzi, G. and Rizzoni, G. and Taylor, C. M. and Van de Kar, N. and Zimmerhackl, L. B.},
  issn         = {1523-1755},
  language     = {eng},
  number       = {3},
  pages        = {423--431},
  publisher    = {Nature Publishing Group},
  series       = {Kidney International},
  title        = {A classification of hemolytic uremic syndrome and thrombotic thrombocytopenic purpura and related disorders},
  url          = {http://dx.doi.org/10.1038/sj.ki.5001581},
  doi          = {10.1038/sj.ki.5001581},
  volume       = {70},
  year         = {2006},
}