Haemolytic uraemic syndrome
Karpman, Diana LU
; Loos, Sebastian
LU
; Tati, Ramesh
LU
and Arvidsson, Ida
LU
(2017)
In Journal of Internal Medicine
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.
(Less)
- author
- Karpman, Diana
LU
; Loos, Sebastian
LU
; Tati, Ramesh
LU
and Arvidsson, Ida
LU
- organization
- publishing date
- 2017
- type
- Contribution to journal
- publication status
- published
- subject
- keywords
- Complement, Enterohaemorrhagic Escherichia coli, Haemolytic uraemic syndrome, Microvesicles, Shiga toxin
- in
- Journal of Internal Medicine
- volume
- 281
- issue
- 2
- pages
- 123 - 148
- publisher
- Wiley-Blackwell
- external identifiers
-
- scopus:84990967215
- pmid:27723152
- 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
- 2024-04-19 11:56:32
@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}},
keywords = {{Complement; Enterohaemorrhagic Escherichia coli; Haemolytic uraemic syndrome; Microvesicles; Shiga toxin}},
language = {{eng}},
number = {{2}},
pages = {{123--148}},
publisher = {{Wiley-Blackwell}},
series = {{Journal of Internal Medicine}},
title = {{Haemolytic uraemic syndrome}},
url = {{http://dx.doi.org/10.1111/joim.12546}},
doi = {{10.1111/joim.12546}},
volume = {{281}},
year = {{2017}},
}