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Consensus regarding diagnosis and management of atypical hemolytic uremic syndrome

Authors
 Hajeong Lee  ;  Eunjeong Kang  ;  Hee Gyung Kang  ;  Young Hoon Kim  ;  Jin Seok Kim  ;  Hee-Jin Kim  ;  Kyung Chul Moon  ;  Tae Hyun Ban  ;  Se Won Oh  ;  Sang Kyung Jo  ;  Heeyeon Cho  ;  Bum Soon Choi  ;  Junshik Hong  ;  Hae Il Cheong  ;  Doyeun Oh 
Citation
 KOREAN JOURNAL OF INTERNAL MEDICINE, Vol.35(1) : 25-40, 2020-01 
Journal Title
KOREAN JOURNAL OF INTERNAL MEDICINE
ISSN
 1226-3303 
Issue Date
2020-01
MeSH
Atypical Hemolytic Uremic Syndrome* / diagnosis ; Atypical Hemolytic Uremic Syndrome* / therapy ; Complement Inactivating Agents / therapeutic use ; Consensus ; Diagnosis, Differential ; Female ; Humans ; Pregnancy ; Thrombotic Microangiopathies* / diagnosis
Keywords
Thrombotic microangiopathies ; Atypical hemolytic uremic syndrome ; Complement pathway ; alternative ; Diagnosis ; differential ; Eculizumab
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:
T9992020542.pdf Download
DOI
10.3904/kjim.2019.388
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Internal Medicine (내과학교실) > 1. Journal Papers
Yonsei Authors
Kim, Jin Seok(김진석) ORCID logo https://orcid.org/0000-0001-8986-8436
URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/190317
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