Consensus regarding diagnosis and management of atypical hemolytic uremic syndromeopen access
- Authors
- Lee, Hajeong; Kang, Eunjeong; Kang, Hee Gyung; Kim, Young Hoon; Kim, Jin Seok; Kim, Hee-Jin; Moon, Kyung Chul; Ban, Tae Hyun; Oh, Se Won; Jo, Sang Kyung; Cho, Heeyeon; Choi, Bum Soon; Hong, Junshik; Cheong, Hae Il; Oh, Doyeun
- Issue Date
- Jan-2020
- Publisher
- 대한내과학회
- Keywords
- Thrombotic microangiopathies; Atypical hemolytic uremic syndrome; Complement pathway; alternative; Diagnosis; differential; Eculizumab
- Citation
- The Korean Journal of Internal Medicine, v.35, no.1, pp 25 - 40
- Pages
- 16
- Indexed
- SCIE
SCOPUS
KCI
- Journal Title
- The Korean Journal of Internal Medicine
- Volume
- 35
- Number
- 1
- Start Page
- 25
- End Page
- 40
- URI
- https://scholarworks.korea.ac.kr/kumedicine/handle/2020.sw.kumedicine/1145
- DOI
- 10.3904/kjim.2019.388
- ISSN
- 1226-3303
2005-6648
- Abstract
- Thrombotic microangiopathy (TMA) is defined by specific clinical characteristics, including microangiopathic hemolytic anemia, thrombocytopenia, and pathologic evidence of endothelial cell damage, as well as the resulting ischemic end-organ injuries. A variety of clinical scenarios have features of TMA, including infection, pregnancy, malignancy, autoimmune disease, and medications. These overlapping manifestations hamper differential diagnosis of the underlying pathogenesis, despite recent advances in understanding the mechanisms of several types of TMA syndrome. Atypical hemolytic uremic syndrome (aHUS) is caused by a genetic or acquired defect in regulation of the alternative complement pathway. It is important to consider the possibility of aHUS in all patients who exhibit TMA with triggering conditions because of the incomplete genetic penetrance of aHUS. Therapeutic strategies for aHUS are based on functional restoration of the complement system. Eculizumab, a monoclonal antibody against the terminal complement component 5 inhibitor, yields good outcomes that include prevention of organ damage and premature death. However, there remain unresolved challenges in terms of treatment duration, cost, and infectious complications. A consensus regarding diagnosis and management of TMA syndrome would enhance understanding of the disease and enable treatment decision-making.
- Files in This Item
- There are no files associated with this item.
- Appears in
Collections - 2. Clinical Science > Department of Nephrology and Hypertension > 1. Journal Articles
Items in ScholarWorks are protected by copyright, with all rights reserved, unless otherwise indicated.