Typical and Atypical Hemolytic Uremic Syndrome in the Critically Ill

Crit Care Clin. 2020 Apr;36(2):333-356. doi: 10.1016/j.ccc.2019.11.004. Epub 2020 Jan 31.


Hemolytic uremic syndrome is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. Disseminated intravascular coagulation, thrombotic thrombocytopenic purpura, and hemolytic uremic syndrome have a similar clinical presentation. Diagnostic needs to be prompt to decrease mortality, because identifying the different disorders can help to tailor specific, effective therapies. However, diagnosis is challenging and morbidity and mortality remain high, especially in the critically ill population. Development of clinical prediction scores and rapid diagnostic tests for hemolytic uremic syndrome based on mechanistic knowledge are needed to facilitate early diagnosis and assign timely specific treatments to patients with hemolytic uremic syndrome variants.

Keywords: Atypical HUS; Complement-mediated HUS; Critically ill; Microangiopathy.

Publication types

  • Review

MeSH terms

  • Algorithms
  • Atypical Hemolytic Uremic Syndrome / diagnosis
  • Atypical Hemolytic Uremic Syndrome / etiology
  • Atypical Hemolytic Uremic Syndrome / genetics
  • Atypical Hemolytic Uremic Syndrome / therapy
  • Critical Illness*
  • Diagnosis, Differential
  • Early Diagnosis
  • Hemolytic-Uremic Syndrome / diagnosis*
  • Hemolytic-Uremic Syndrome / etiology*
  • Hemolytic-Uremic Syndrome / genetics
  • Hemolytic-Uremic Syndrome / therapy
  • Humans
  • Prognosis
  • Risk Factors
  • Shiga Toxin / toxicity


  • Shiga Toxin