0 111

Cited 8 times in

Clinical Features of Hereditary Angioedema in Korean Patients: A Nationwide Multicenter Study

Authors
 Jung J.-W.  ;  Suh D.I.  ;  Park H.J.  ;  Kim S.  ;  Kwon H. S.  ;  Yang M. S.  ;  Park C.S.  ;  Kim J.-H.  ;  Kim S.-H.  ;  Lee Y.W.  ;  Hur G.Y.  ;  Ye Y.-M.  ;  Kwon Y.E.  ;  Park H.-K.  ;  Kim C.W.  ;  Koh Y.-I.  ;  Park J.W.  ;  Lee J.-M.  ;  Min K.-U.  ;  Wickner P.  ;  Kang H.-R. 
Citation
 INTERNATIONAL ARCHIVES OF ALLERGY AND IMMUNOLOGY, Vol.176(3~4) : 272-279, 2018 
Journal Title
 INTERNATIONAL ARCHIVES OF ALLERGY AND IMMUNOLOGY 
ISSN
 1018-2438 
Issue Date
2018
MeSH
Adult ; Angioedemas, Hereditary/complications* ; Angioedemas, Hereditary/diagnosis ; Angioedemas, Hereditary/drug therapy ; Danazol/therapeutic use ; Delayed Diagnosis ; Female ; Humans ; Male ; Middle Aged ; Republic of Korea ; Retrospective Studies
Keywords
Angioedema ; Complement C1s esterase inhibitor proteins ; Hereditary angioedema types I and II ; Koreans ; Mortality
Abstract
BACKGROUND: Hereditary angioedema (HAE) is a genetically heterogeneous autosomal dominant disorder characterized by recurrent episodes of nonpruritic, nonpitting edema increasing after puberty. It can be fatal due to laryngeal or gastrointestinal (GI) involvement with varied and changing frequency of mortality according to studies published from the Western countries. Epidemiological and clinical data of HAE in Asian countries are sparse. We sought to examine the clinical characteristics of HAE patients in Korea. METHODS: Patients diagnosed with HAE at 15 tertiary hospitals across the country until 2016 were retrospectively reviewed. RESULTS: A total of 65 patients diagnosed with HAE by 2016 were identified. The prevalence of HAE was estimated at 1.3/1,000,000 in Korea. Of the 65 patients, 21 (32.3%) were males. A total of 90.8% patients had type I HAE, while the remaining 9.2% patients had type II HAE. The first symptom developed after 20 years in 73.8% of patients, with a mean age 28.4 ± 14.1 years. The age at diagnosis was 36.5 ± 15.8 years, with a mean time delay of 7.8 ± 10.5 years. While the face (82.3%) and extremities (upper 71.0%, lower 62.9%) were the most frequently involved, the GI tract was affected in 40.5% of Korean HAE patients. Prophylaxis was maintained in 62.5% of patients. There was no reported case of death from HAE so far. CONCLUSIONS: The clinical manifestation and severity of HAE may vary according to ethnicity. HAE is more infrequent and GI involvement is less likely in Korea compared with Western countries.
Full Text
https://www.karger.com/Article/FullText/488350
DOI
10.1159/000488350
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Internal Medicine (내과학교실) > 1. Journal Papers
Yonsei Authors
Park, Hye Jung(박혜정) ORCID logo https://orcid.org/0000-0002-1862-1003
URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/167160
사서에게 알리기
  feedback

qrcode

Items in DSpace are protected by copyright, with all rights reserved, unless otherwise indicated.

Browse

Links