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Author: Dr Rebecca Irwin, Junior Registrar, Basic Medicine Trainee, Waikato Hospital, Hamilton, New Zealand. Copy edited by Gus Mitchell. July 2021
Haemolytic uraemic syndrome (HUS), a form of thrombotic microangiopathy, is defined by the clinical triad of thrombocytopenia, anaemia, and acute kidney damage.
Haemolytic uraemic syndrome is one of the leading causes of acute kidney damage in children and is becoming increasingly common in adults.
The pathophysiology of HUS involves vascular endothelial cell damage which increases the risk of thrombosis and organ damage, particularly of the kidneys. Disorders of the complement system may predispose to the development of HUS in response to specific triggers, with the resultant dysregulation of the complement cascade.
Haemolytic uraemic syndrome can be classified as:
Haemolytic uraemic syndrome presents with:
Diarrhoea-associated haemolytic uraemic syndrome may be complicated by:
Neurological complications of HUS include stroke and coma.
Haemolytic uraemic syndrome is diagnosed on the following findings:
The cause and complications of HUS should be investigated.
Haemolytic uraemic syndrome can cause acute and/or end-stage renal failure. Most patients with Shiga toxin-related HUS recover fully. Poor prognostic signs include neurological symptoms, low neutrophil count, low platelet count, and prolonged anuria.
The prognosis for atypical HUS depends on the associated trigger. Eculizumab, if available, has improved outcomes.
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