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Haemolytic uraemic syndrome

Karpman, Diana LU ; Loos, Sebastian LU ; Tati, Ramesh LU and Arvidsson, Ida LU (2017) In Journal of Internal Medicine1989-01-01+01:00 281(2). p.123-148
Abstract

Haemolytic uraemic syndrome (HUS) is defined by the simultaneous occurrence of nonimmune haemolytic anaemia, thrombocytopenia and acute renal failure. This leads to the pathological lesion termed thrombotic microangiopathy, which mainly affects the kidney, as well as other organs. HUS is associated with endothelial cell injury and platelet activation, although the underlying cause may differ. Most cases of HUS are associated with gastrointestinal infection with Shiga toxin-producing enterohaemorrhagic Escherichia coli (EHEC) strains. Atypical HUS (aHUS) is associated with complement dysregulation due to mutations or autoantibodies. In this review, we will describe the causes of HUS. In addition, we will review the clinical,... (More)

Haemolytic uraemic syndrome (HUS) is defined by the simultaneous occurrence of nonimmune haemolytic anaemia, thrombocytopenia and acute renal failure. This leads to the pathological lesion termed thrombotic microangiopathy, which mainly affects the kidney, as well as other organs. HUS is associated with endothelial cell injury and platelet activation, although the underlying cause may differ. Most cases of HUS are associated with gastrointestinal infection with Shiga toxin-producing enterohaemorrhagic Escherichia coli (EHEC) strains. Atypical HUS (aHUS) is associated with complement dysregulation due to mutations or autoantibodies. In this review, we will describe the causes of HUS. In addition, we will review the clinical, pathological, haematological and biochemical features, epidemiology and pathogenetic mechanisms as well as the biochemical, microbiological, immunological and genetic investigations leading to diagnosis. Understanding the underlying mechanisms of the different subtypes of HUS enables tailoring of appropriate treatment and management. To date, there is no specific treatment for EHEC-associated HUS but patients benefit from supportive care, whereas patients with aHUS are effectively treated with anti-C5 antibody to prevent recurrences, both before and after renal transplantation.

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author
organization
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type
Contribution to journal
publication status
published
subject
keywords
Complement, Enterohaemorrhagic Escherichia coli, Haemolytic uraemic syndrome, Microvesicles, Shiga toxin
in
Journal of Internal Medicine1989-01-01+01:00
volume
281
issue
2
pages
123 - 148
publisher
Wiley-Blackwell Publishing Ltd
external identifiers
  • scopus:84990967215
  • wos:000393950900002
ISSN
0954-6820
DOI
10.1111/joim.12546
language
English
LU publication?
yes
id
c77f5c39-a8da-41dc-a0df-bdb286dcf755
date added to LUP
2016-11-08 11:53:34
date last changed
2018-01-07 11:34:12
@article{c77f5c39-a8da-41dc-a0df-bdb286dcf755,
  abstract     = {<p>Haemolytic uraemic syndrome (HUS) is defined by the simultaneous occurrence of nonimmune haemolytic anaemia, thrombocytopenia and acute renal failure. This leads to the pathological lesion termed thrombotic microangiopathy, which mainly affects the kidney, as well as other organs. HUS is associated with endothelial cell injury and platelet activation, although the underlying cause may differ. Most cases of HUS are associated with gastrointestinal infection with Shiga toxin-producing enterohaemorrhagic Escherichia coli (EHEC) strains. Atypical HUS (aHUS) is associated with complement dysregulation due to mutations or autoantibodies. In this review, we will describe the causes of HUS. In addition, we will review the clinical, pathological, haematological and biochemical features, epidemiology and pathogenetic mechanisms as well as the biochemical, microbiological, immunological and genetic investigations leading to diagnosis. Understanding the underlying mechanisms of the different subtypes of HUS enables tailoring of appropriate treatment and management. To date, there is no specific treatment for EHEC-associated HUS but patients benefit from supportive care, whereas patients with aHUS are effectively treated with anti-C5 antibody to prevent recurrences, both before and after renal transplantation.</p>},
  author       = {Karpman, Diana and Loos, Sebastian and Tati, Ramesh and Arvidsson, Ida},
  issn         = {0954-6820},
  keyword      = {Complement,Enterohaemorrhagic Escherichia coli,Haemolytic uraemic syndrome,Microvesicles,Shiga toxin},
  language     = {eng},
  number       = {2},
  pages        = {123--148},
  publisher    = {Wiley-Blackwell Publishing Ltd},
  series       = {Journal of Internal Medicine1989-01-01+01:00},
  title        = {Haemolytic uraemic syndrome},
  url          = {http://dx.doi.org/10.1111/joim.12546},
  volume       = {281},
  year         = {2017},
}