Haemolytic Uraemic Syndrome (HUS)



E Coli
E. coli

Background

  • Haemolytic uraemic syndrome (HUS) - progressive failure that is associated with microangiopathic haemolytic anaemia and thrombocytopenia.
  • Variable signs and symptoms of end-organ ischaemia.
  • M = F
  • Infective HUS (also known as diarrhoea-associated HUS or D+HUS) is recognised as a sequel to infection primarily with two gastrointestinal pathogens: Verocytoxigenic Escherichia coli (VTEC1) and Shigella dysenteriae.
  • Verocytotoxigenic Escherichia coli (VTEC1), or the pathogen responsible for the large outbreak of gastroenteritis in Germany in 2011 that produced 4000 cases of illness, of which more than 800 developed haemolytic uraemic Syndrome (HUS) and 50 died.
  • VTEC1 is highly transmissible; it can be spread from person‐to‐person, through food and water and from surfaces.
  • The infectious dose is tiny.
  • Ireland has currently (Jan 2013) the highest notification rate of VTEC (incidence rate of 12.16/100,000) in Europe with a high incidence of HUS (10%). 3% of HUS cases die. All of these HUS cases were admitted to hospital for extensive supportive measures.
  • HUS is the commonest cause of acute renal failure in children.
  • Severity of the acute illness, especially the presence of CNS symptoms, is strongly associated with poorer long‐term outcome.

Clinical

Triad of:

Presentation

  • ↓ urinary output
  • Weakness, Lethargy
  • Pallor
  • Purpura
  • Epistaxis
  • Oedema

Differential Dx


Investigations

  • ARF (↑↑ Urea & Creat)
  • Thrombocytopenia with normal PT / APTT
  • Urine +ve for prot / RBC
  • D-Dimers Normal
  • Retic
  • Blood cultures Negative (toxic causes disease, not bugs)
  • Stool cultures positive
    • Stool MC&S if:
      • Features suggestive of HUS

Management

  • Aggressive fluid resuscitation
  • ± correction U&E abnormality
  • Urgent nephrological opinion
  • Admit paediatrics
  • Notify public health


Content By Dr Íomhar O' Sullivan date. Last review Dr ÍOS 5/12/18.