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A Case of Portopulmonary Hypertension Associated with Primary Biliary Cirrhosis  

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)
Publication Information
Tuberculosis and Respiratory Diseases / v.62, no.5, 2007 , pp. 421-426 More about this Journal
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.
Keywords
Portal hypertension; Primary biliary cirrhosis; Pulmonary hypertension;
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