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

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

Author information

1
Department of Pediatrics, Seoul National University Children's Hospital, Seoul, Korea.
2
Research Coordination Center for Rare Diseases, Seoul National University Hospital, Seoul, Korea.
3
Division of Nephrology, Department of Internal Medicine, Korea University School of Medicine, Seoul, Korea.
4
Department of Internal Medicine, Seoul National University, Seoul, Korea.
5
Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
6
Division of Hematology, Department of Internal Medicine, Yonsei University College of Medicine, Severance Hospital, Seoul, Korea.
7
Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Uijeongbu, Korea.
8
Division of Nephrology, Dongtan Sacred Heart Hospital, Hallym University Medical Center, Hwaseong, Korea.
9
Division of Hematology, Department of Internal Medicine, Korea University School of Medicine, Seoul, Korea.
10
Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Korea.
11
Department of Pediatrics, Severance Children's Hospital, Yonsei University College of Medicine, Seoul, Korea.
12
Department of Pediatrics, Korea University Guro Hospital, Seoul, Korea.
13
Department of Internal Medicine, School of Medicine, CHA University, Seongnam, Korea. doh@cha.ac.kr.

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.

KEYWORDS:

Atypical Hemolytic Uremic Syndrome; Diagnosis; Guidelines; Treatment

PMID:
27550478
PMCID:
PMC4999392
DOI:
10.3346/jkms.2016.31.10.1516
[Indexed for MEDLINE]
Free PMC Article

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