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Clinical features

Authoring team

Clinical features include:

  • variceal bleeding - 80% of patients present between 1 and 6 years of age; in 70% of which, there is a preceding upper respiratory tract infection
  • splenomegaly
  • pancytopenia
  • splenectomy - may have been advised earlier because of the bleeding and a failure to recognise the underlying problem
  • increased frequency of neuropsychiatric problems - a subtle form of encephalopathy

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