Clinical Practice Guidelines for the Management of Atypical Hemolytic Uremic Syndrome in Korea

J Korean Med Sci. 2016 Oct;31(10):1516-28. doi: 10.3346/jkms.2016.31.10.1516.


Atypical hemolytic uremic syndrome (aHUS) is a rare syndrome characterized by micro-angiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. The major pathogenesis of aHUS involves dysregulation of the complement system. Eculizumab, which blocks complement C5 activation, has recently been proven as an effective agent. Delayed diagnosis and treatment of aHUS can cause death or end-stage renal disease. Therefore, a diagnosis that differentiates aHUS from other forms of thrombotic microangiopathy is very important for appropriate management. These guidelines aim to offer recommendations for the diagnosis and treatment of patients with aHUS in Korea. The guidelines have largely been adopted from the current guidelines due to the lack of evidence concerning the Korean population.

Keywords: Atypical Hemolytic Uremic Syndrome; Diagnosis; Guidelines; Treatment.

Publication types

  • Practice Guideline

MeSH terms

  • ADAMTS13 Protein / genetics
  • ADAMTS13 Protein / metabolism
  • Acute Kidney Injury / etiology
  • Antibodies, Monoclonal, Humanized / therapeutic use*
  • Atypical Hemolytic Uremic Syndrome / diagnosis*
  • Atypical Hemolytic Uremic Syndrome / epidemiology
  • Atypical Hemolytic Uremic Syndrome / therapy
  • Blood Transfusion*
  • Complement Factor H / genetics
  • Complement System Proteins / genetics
  • Complement System Proteins / metabolism
  • Delayed Diagnosis
  • Humans
  • Kidney Failure, Chronic / etiology
  • Organ Transplantation*
  • Republic of Korea / epidemiology


  • Antibodies, Monoclonal, Humanized
  • Complement Factor H
  • Complement System Proteins
  • eculizumab
  • ADAMTS13 Protein