J Korean Med Sci.  2016 Oct;31(10):1516-1528. 10.3346/jkms.2016.31.10.1516.

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

• Cheong HI
• Jo SK
• Yoon SS
• Cho H
• Kim JS
• Kim YO
• Koo JR
• Park Y
• Park YS
• Shin JI
• Yoo KH
• Oh D

• KMID: 2468240
• DOI: http://doi.org/10.3346/jkms.2016.31.10.1516

Abstract

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.

Keyword

Guidelines; Diagnosis; Treatment; Atypical Hemolytic Uremic Syndrome

MeSH Terms

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

Figure

• 

  Fig. 1 Etiology of atypical hemolytic uremic syndrome (aHUS) in a Korean pediatric cohort.

• 

  Fig. 2 Proposed diagram to differentiate atypical hemolytic uremic syndrome (aHUS) among thrombotic micro-angiopathies. MAHA, microangiopathic haemolytic anaemia; TMA, thrombotic microangiopathy; DIC, disseminated intravascular coagulation; HIT, heparin-induced thrombocytopenia; CAPS, Catastrophic antiphospholipid syndrome; HELLP, hemolysis, elevated liver enzymes, and low platelet count syndrome; PNH, paroxysmal nocturnal hemoglobinuria; VEGF, vascular endothelium growth factor; U/A, urine analysis; HIV, human immunodeficiency virus; ADAMTS, A Disintegrin And Metalloproteinase with a ThromboSpondin type 1 motif, member 13; EHEC, enterohemorrhagic Escherichia coli; STEC, Shiga toxin-producing Escherichia coli; HUS, hemolytic uremic symdrome; TTP, thrombotic thrombocytopenic purpura; aHUS, atypical hemolytic uremic symdrome.

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