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Haemolytic Uraemic Syndrome

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General Points

  1. Haemolytic-uraemic syndrome (HUS) is an important cause of acute renal failure in children
  2. 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.
  3. Diarrhoea associated outbreak cases and atypical cases have a higher incidence of acute and chronic renal failure and a higher mortality.
  4. 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.
  5. Mortality is 2-6.6% and incidence of acute renal failure requiring renal replacement therapy is ~50%.
  6. 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.
  7. 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.
  8. 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.
  9. With early diagnosis and good supportive management the majority of cases with typical HUS do well and have no long term morbidity.

Investigations

  1. Full blood count with film. Anaemia, thromboycytopenia and a blood film with red blood cell fragmentation is typical.
  2. Urea, creatinine and electrolytes.
  3. An accurate fluid balance.
  4. Regular neurological assessment. Consider CT head and EEG in cases of decreased level of consciousness and or seizures.
  5. Stool sample for analysis for verotoxin-producing E. coli.

Management

  1. Management of HUS is supportive.
  2. 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.
  3. See acute renal failure and hypertension protocols for treatment of these problems.

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Document Control

  • 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