Haemolytic uraemic syndrome

Diana Karpman; Sebastian Loos; Ramesh Tati; Ida Arvidsson

doi:10.1111/joim.12546

Haemolytic uraemic syndrome

Standard

Haemolytic uraemic syndrome. / Karpman, Diana; Loos, Sebastian; Tati, Ramesh; Arvidsson, Ida.

In: J INTERN MED, Vol. 281, No. 2, 02.2017, p. 123-148.

Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review

Harvard

Karpman, D, Loos, S, Tati, R & Arvidsson, I 2017, 'Haemolytic uraemic syndrome', J INTERN MED, vol. 281, no. 2, pp. 123-148. https://doi.org/10.1111/joim.12546

APA

Karpman, D., Loos, S., Tati, R., & Arvidsson, I. (2017). Haemolytic uraemic syndrome. J INTERN MED, 281(2), 123-148. https://doi.org/10.1111/joim.12546

Vancouver

Karpman D, Loos S, Tati R, Arvidsson I. Haemolytic uraemic syndrome. J INTERN MED. 2017 Feb;281(2):123-148. https://doi.org/10.1111/joim.12546

Bibtex

@article{8bbc29a0afee446b8970fa8b19c68cb8,

title = "Haemolytic uraemic syndrome",

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, 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.",

author = "Diana Karpman and Sebastian Loos and Ramesh Tati and Ida Arvidsson",

note = "{\textcopyright} 2016 The Association for the Publication of the Journal of Internal Medicine.",

year = "2017",

month = feb,

doi = "10.1111/joim.12546",

language = "English",

volume = "281",

pages = "123--148",

journal = "J INTERN MED",

issn = "0954-6820",

publisher = "Wiley-Blackwell",

number = "2",

}

RIS

TY - JOUR

T1 - Haemolytic uraemic syndrome

AU - Karpman, Diana

AU - Loos, Sebastian

AU - Tati, Ramesh

AU - Arvidsson, Ida

PY - 2017/2

Y1 - 2017/2

N2 - 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.

AB - 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.

U2 - 10.1111/joim.12546

DO - 10.1111/joim.12546

M3 - SCORING: Journal article

C2 - 27723152

VL - 281

SP - 123

EP - 148

JO - J INTERN MED

JF - J INTERN MED

SN - 0954-6820

IS - 2

ER -