A Case of Portopulmonary Hypertension Associated with Primary Biliary Cirrhosis / 결핵및호흡기질환
Tuberculosis and Respiratory Diseases
; : 421-426, 2007.
Article
in Korean
| WPRIM (Western Pacific)
| ID: wpr-121714
Responsible library:
WPRO
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.
Full text:
Available
Database:
WPRIM (Western Pacific)
Main subject:
Pulmonary Artery
/
Thrombosis
/
Vasoconstriction
/
Dyspnea
/
Endothelium
/
Hypertension
/
Hypertension, Portal
/
Hypertension, Pulmonary
/
Liver Cirrhosis, Biliary
/
Liver Diseases
Limits:
Female
/
Humans
Language:
Korean
Journal:
Tuberculosis and Respiratory Diseases
Year:
2007
Document type:
Article