title: Clinical Practice Guidelines for the Management of Atypical Hemolytic Uremic Syndrome in Korea.
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.
authors.affiliation: Department of Pediatrics, Seoul National University Children's Hospital, Seoul, Korea.|Research Coordination Center for Rare Diseases, Seoul National University Hospital, Seoul, Korea.
authors.forename: Hae Il
authors.identifier: http://orcid.org/0000-0001-7556-1265
authors.initials: HI
authors.lastname: Cheong
authors.affiliation: Division of Nephrology, Department of Internal Medicine, Korea University School of Medicine, Seoul, Korea.
authors.forename: Sang Kyung
authors.identifier: http://orcid.org/0000-0002-0496-0258
authors.initials: SK
authors.lastname: Jo
authors.affiliation: Department of Internal Medicine, Seoul National University, Seoul, Korea.
authors.forename: Sung Soo
authors.identifier: http://orcid.org/0000-0003-2591-7459
authors.initials: SS
authors.lastname: Yoon
authors.affiliation: Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
authors.forename: Heeyeon
authors.identifier: http://orcid.org/0000-0003-3137-6054
authors.initials: H
authors.lastname: Cho
authors.affiliation: Division of Hematology, Department of Internal Medicine, Yonsei University College of Medicine, Severance Hospital, Seoul, Korea.
authors.forename: Jin Seok
authors.identifier: http://orcid.org/0000-0001-8986-8436
authors.initials: JS
authors.lastname: Kim
authors.affiliation: Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Uijeongbu, Korea.
authors.forename: Young Ok
authors.identifier: http://orcid.org/0000-0001-8131-7998
authors.initials: YO
authors.lastname: Kim
authors.affiliation: Division of Nephrology, Dongtan Sacred Heart Hospital, Hallym University Medical Center, Hwaseong, Korea.
authors.forename: Ja Ryong
authors.identifier: http://orcid.org/0000-0003-4245-2569
authors.initials: JR
authors.lastname: Koo
authors.affiliation: Division of Hematology, Department of Internal Medicine, Korea University School of Medicine, Seoul, Korea.
authors.forename: Yong
authors.identifier: http://orcid.org/0000-0001-7909-6639
authors.initials: Y
authors.lastname: Park
authors.affiliation: Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Korea.
authors.forename: Young Seo
authors.identifier: http://orcid.org/0000-0001-7653-2036
authors.initials: YS
authors.lastname: Park
authors.affiliation: Department of Pediatrics, Severance Children's Hospital, Yonsei University College of Medicine, Seoul, Korea.
authors.forename: Jae Il
authors.identifier: http://orcid.org/0000-0003-2326-1820
authors.initials: JI
authors.lastname: Shin
authors.affiliation: Department of Pediatrics, Korea University Guro Hospital, Seoul, Korea.
authors.forename: Kee Hwan
authors.identifier: http://orcid.org/0000-0001-6490-4293
authors.initials: KH
authors.lastname: Yoo
authors.affiliation: Department of Internal Medicine, School of Medicine, CHA University, Seongnam, Korea. doh@cha.ac.kr.
authors.forename: Doyeun
authors.identifier: http://orcid.org/0000-0002-6981-3144
authors.initials: D
authors.lastname: Oh
mesh_terms: D000071120:ADAMTS13 Protein / Q000235:genetics / Q000378:metabolism; D058186:Acute Kidney Injury / Q000209:etiology; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use*; D065766:Atypical Hemolytic Uremic Syndrome / Q000175:diagnosis* / Q000453:epidemiology / Q000628:therapy; D001803:Blood Transfusion; D017242:Complement Factor H / Q000235:genetics; D003165:Complement System Proteins / Q000235:genetics / Q000378:metabolism; D057210:Delayed Diagnosis; D006801:Humans; D007676:Kidney Failure, Chronic / Q000209:etiology; D016377:Organ Transplantation; D056910:Republic of Korea / Q000453:epidemiology
chemical_list: D061067:Antibodies, Monoclonal, Humanized; D017242:Complement Factor H; D003165:Complement System Proteins; C481642:eculizumab; D000071120:ADAMTS13 Protein
keywords: Atypical Hemolytic Uremic Syndrome; Diagnosis; Guidelines; Treatment