Tuberculosis and Respiratory Diseases

The Korean Academy of Tuberculosis and Respiratory Diseases (대한결핵및호흡기학회)
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A Case of Portopulmonary Hypertension Associated with Primary Biliary Cirrhosis

원발성 담관성 간경변과 동반된 문맥폐고혈압 1예

  • Kim, Se Joong (Department of Internal Medicine, College of Medicine, Korea University) ;
  • Lee, Eun Ju (Department of Internal Medicine, College of Medicine, Korea University) ;
  • Jung, Ki Hwan (Department of Internal Medicine, College of Medicine, Korea University) ;
  • Kang, Eun Hae (Department of Internal Medicine, College of Medicine, Korea University) ;
  • Lee, Sung Yong (Department of Internal Medicine, College of Medicine, Korea University) ;
  • Lim, Hong Euy (Department of Internal Medicine, College of Medicine, Korea University) ;
  • Yim, Hyung Joon (Department of Internal Medicine, College of Medicine, Korea University) ;
  • Lee, Sang Yeub (Department of Internal Medicine, College of Medicine, Korea University) ;
  • Kim, Je Hyeong (Department of Internal Medicine, College of Medicine, Korea University) ;
  • Shin, Chol (Department of Internal Medicine, College of Medicine, Korea University) ;
  • Shim, Jae Jeong (Department of Internal Medicine, College of Medicine, Korea University) ;
  • In, Kwang Ho (Department of Internal Medicine, College of Medicine, Korea University) ;
  • Kang, Kyung Ho (Department of Internal Medicine, College of Medicine, Korea University) ;
  • Yoo, Se Hwa (Department of Internal Medicine, College of Medicine, Korea University)
  • 김세중 (고려대학교 의과대학 내과학교실) ;
  • 이은주 (고려대학교 의과대학 내과학교실) ;
  • 정기환 (고려대학교 의과대학 내과학교실) ;
  • 강은해 (고려대학교 의과대학 내과학교실) ;
  • 이승룡 (고려대학교 의과대학 내과학교실) ;
  • 임홍의 (고려대학교 의과대학 내과학교실) ;
  • 임형준 (고려대학교 의과대학 내과학교실) ;
  • 이상엽 (고려대학교 의과대학 내과학교실) ;
  • 김제형 (고려대학교 의과대학 내과학교실) ;
  • 신철 (고려대학교 의과대학 내과학교실) ;
  • 심재정 (고려대학교 의과대학 내과학교실) ;
  • 인광호 (고려대학교 의과대학 내과학교실) ;
  • 강경호 (고려대학교 의과대학 내과학교실) ;
  • 유세화 (고려대학교 의과대학 내과학교실)
  • Received : 2007.03.09
  • Accepted : 2007.05.02
  • Published : 2007.05.30
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Abstract

Portopulmonary hypertension (PPHTN) is a clinically and pathophysiologically distinct complication of advanced liver disease. PPHTN is characterized by the development of pulmonary arterial hypertension in association with advanced hepatic disease-related portal hypertension. A characteristic feature of PPHTN is an obstruction to the pulmonary artery flow caused by vasoconstriction, the proliferation of the endothelium and smooth muscle components of the vascular wall, as well as in situ thrombosis. This disorder is commonly underdiagnosed but the clinical implications are significant because it has substantial effects on survival and requires special treatment. We report a case of portopulmonary hypertension in a 53-year-old woman with primary biliary cirrhosis who presented with exertional dyspnea.

진행성 간질환의 합병증으로 발생하는 문맥폐고혈압은, 이차성 폐동맥고혈압 중에서 진행성 간질환에 의한 문맥고혈압과 연관된 폐동맥고혈압으로 분류되며, 독특한 임상적, 병태생리학적 특징을 보인다. 임상양상은 점진적인 운동성 호흡곤란, 흉통 등을 호소하지만, 증상 없이 우연히 발견되는 경우도 있다. 심초음파 검사를 통해 예비적으로, 우심장 도관삽입으로 직접 우심실 및 평균 폐동맥압을 측정하여 진단할 수 있다. 치료는 칼슘통로차단제, 프로스타노이드 등을 사용하지만, 근본적인 치료를 위해서는 간 및 폐이식을 고려해야 한다. 저자들은 53세 여자로 20년 전에 간경변을 진단받은 후 호흡곤란으로 내원한 환자에게서, 간경변의 원인으로 원발성 담관성 간경변을 진단하고, 호흡곤란의 원인으로 문맥폐고혈압을 진단하여 치료하였기에 문헌고찰과 함께 보고한다.

Keywords

References

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