The Korean Journal of Medicine

  • 제80권5호
  • /
  • Pages.578-582
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  • 2011
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  • 1738-9364(pISSN)
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  • 2289-0769(eISSN)
대한내과학회 (The Korean Association of Internal Medicine)
권호 표지

특발성 간경변증을 동반한 POEMS 증후군 1예

A Case of POEMS Syndrome Associated with Idiopathic Liver Cirrhosis

  • 고광훈 (충남대학교 의학전문대학원 내과학교실) ;
  • 정재훈 (충남대학교 의학전문대학원 내과학교실) ;
  • 문희석 (충남대학교 의학전문대학원 내과학교실) ;
  • 성재규 (충남대학교 의학전문대학원 내과학교실) ;
  • 김석현 (충남대학교 의학전문대학원 내과학교실) ;
  • 이병석 (충남대학교 의학전문대학원 내과학교실) ;
  • 이헌영 (충남대학교 의학전문대학원 내과학교실)
  • Ko, Kwang-Hun (Department of Internal Medicine, School of Medicine, Chungnam National University) ;
  • Jung, Jae-Hoon (Department of Internal Medicine, School of Medicine, Chungnam National University) ;
  • Moon, Hee-Seok (Department of Internal Medicine, School of Medicine, Chungnam National University) ;
  • Seong, Jae-Kyu (Department of Internal Medicine, School of Medicine, Chungnam National University) ;
  • Kim, Seok-Hyun (Department of Internal Medicine, School of Medicine, Chungnam National University) ;
  • Lee, Byung-Seok (Department of Internal Medicine, School of Medicine, Chungnam National University) ;
  • Lee, Heon-Young (Department of Internal Medicine, School of Medicine, Chungnam National University)
  • 발행 : 2011.05.01
⟨ 이전 논문 다음 논문 ⟩

초록

POEMS 증후군은 다발성 신경병증(polyneuropathy), 장기종대(organomegaly), 내분비장애(endocrinopathy), 단클론성 감마글로블린병증(monoclonal gammopahty), 피부 병변(skin lesion)을 주 양상으로 발현되는 드문 증후군으로, 본 예에서는 특발성 간경변증을 동반한 POEMS 증후군 환자를 경험하였기에 보고하는 바이다.

POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin lesion) is rare multisystemdisease of unknown cause with varying clinical manifestations. Although there is no established definition for this syndrome,the common classical features are considered to be secondary to the plasma cell dyscrasia with the production of a monoclonalcomponent. Anasarca, including ascites, peripheral edema, and pleural effusion, occurs frequently in POEMS syndrome, butcases with idiopathic liver cirrhosis are rarely reported. We experienced a 73-year-old female with POEMS syndrome whose mainproblem was massive ascites. It is the first report of idiopathic liver cirrhosis in a patient with POEMS syndrome in Korea.

키워드

참고문헌

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