Clinical Practice Guidelines for the Management of Atypical Hemolytic Uremic Syndrome in Korea.
10.3346/jkms.2016.31.10.1516
- Author:
Hae Il CHEONG
1
;
Sang Kyung JO
;
Sung Soo YOON
;
Heeyeon CHO
;
Jin Seok KIM
;
Young Ok KIM
;
Ja Ryong KOO
;
Yong PARK
;
Young Seo PARK
;
Jae Il SHIN
;
Kee Hwan YOO
;
Doyeun OH
Author Information
1. Department of Pediatrics, Seoul National University Children's Hospital, Seoul, Korea.
- Publication Type:Review
- Keywords:
Guidelines;
Diagnosis;
Treatment;
Atypical Hemolytic Uremic Syndrome
- MeSH:
Acute Kidney Injury;
Anemia, Hemolytic;
Atypical Hemolytic Uremic Syndrome*;
Complement C5;
Complement System Proteins;
Delayed Diagnosis;
Diagnosis;
Humans;
Kidney Failure, Chronic;
Korea*;
Thrombocytopenia;
Thrombotic Microangiopathies
- From:Journal of Korean Medical Science
2016;31(10):1516-1528
- CountryRepublic of Korea
- Language:English
-
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.
