Haemolytic Uraemic Syndrome
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- Haemolytic-uraemic syndrome (HUS) is an important cause of acute renal failure in children
- The majority of cases are diarrhoea associated, which may be sporadic or outbreak in nature. A minority of cases are not associated with diarrhoea and are called atypical.
- Diarrhoea associated outbreak cases and atypical cases have a higher incidence of acute and chronic renal failure and a higher mortality.
- Other risk factors associated with an increased morbidity and mortality are severe diarrhoeal prodrome, raised WCC (> 20 x 10E9/L), neurological manifestations and early anuria early.
- Mortality is 2-6.6% and incidence of acute renal failure requiring renal replacement therapy is ~50%.
- The preferred method of renal replacement therapy in HUS is peritoneal dialysis (PD) - see separate protocol, although CVVH should be considered in those with severe disease or CVS instability.
- Other than the renal insufficiency and colitis, HUS is also associated with a number of extra renal manifestations: hypertension, HUS encephalopathy, seizures and pancreatic insufficiency are the ones that are most likely to require clinical input.
- In severe cases, those with atypical HUS, or those whose illness clinically overlaps with thrombotic thrombocytopenic purpura (TTP) there may be a role for plasma exchange or plasma infusion.
- With early diagnosis and good supportive management the majority of cases with typical HUS do well and have no long term morbidity.
- Full blood count with film. Anaemia, thromboycytopenia and a blood film with red blood cell fragmentation is typical.
- Urea, creatinine and electrolytes.
- An accurate fluid balance.
- Regular neurological assessment. Consider CT head and EEG in cases of decreased level of consciousness and or seizures.
- Stool sample for analysis for verotoxin-producing E. coli.
- Management of HUS is supportive.
- Correct dehydration and anaemia (Hb < 70 g/L) if present, and play close attention to fluid balance and electrolytes. Platelet transfusions rarely required and only for clinical bleeding or before surgical procedures such as insertion of a Tenkhoff catheter.
- See acute renal failure and hypertension protocols for treatment of these problems.
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- Date last published: 14 September 2007
- Document type: Clinical Guideline
- Services responsible: Paediatric Intensive Care Unit
- Owner: Gabrielle Nuthall
- Editor: John Beca
- Review frequency: 2 years
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