Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pulmonary arterial hypertension with severe pulmonary vascular disease is a rare association of portal hypertension in man, be it the result of cirrhosis of the liver or obstruction of the portal vein. We induced portal hypertension in 23 rats by partially ligating the portal vein or by totally occluding it in two stages. The rats were killed between one and 15 months after operation. A collateral circulation of varicose, anastomotic vessels was established, and in six animals well-marked oesophageal varices developed. Despite this evidence of sustained portal hypertension, hypertrophy did not develop in the right ventricle or in the media of the pulmonary trunk or muscular pulmonary arteries in any of the animals. This suggests that mechanical obstruction of the portal vein per se is not responsible for the development of pulmonary hypertension. Other factors, perhaps of a humoral nature, appear to be required to induce this rare association of portal hypertension but we have been unable to identify these. In particular, blood levels of oestrogen were not raised after ligation of the portal vein.
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PMID:Observations on the rare association between portal and pulmonary hypertension. 726 72

Pulmonary arterial hypertension PAH is one of the rare expressions of cirrhosis and defined by a pressure more than 30 mmHg. We report our results in a Tunisian Seria results: 21 cirrhotic patients (19M/12F) with a median age of 60 years (22 to 85) explored by cardiac echographic with measures of PAH. We observe an elevated PAH in 31% of cases. Conclusion elevated PAH is relatively frequent during the evolution of cirrhosis. This symptom needs to be systematically explored in patients proposed for liver graft because increased PAH is a contraindication for liver transplantation.
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PMID:[Pulmonary arterial hypertension in cirrhosis]. 1235 40

Pulmonary arterial hypertension is a serious and severe complication of liver cirrhosis and portal hypertension. We present a case of a 47 year old female who developed pulmonary hypertension and right ventricular heart failure symptoms 6 years from the diagnosis of liver biliary cirrhosis and portal hypertension.
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PMID:[Pulmonary arterial hypertension complicating portal hypertension and liver biliary cirrhosis--a case report]. 1650 74

Pulmonary arterial hypertension (PAH) is found in 2-20% of cirrhosis patients who have portal hypertension (portopulmonary hypertension, PPHT). Endothelin (ET), a potent vasoconstrictor, is likely to play a role in the pathogenesis of portal hypertension. We describe the long-term successful use of the dual ET(A)/ET(B) receptor antagonist bosentan in a 43-year-old male with alcohol-related cirrhosis (Child-Pugh A), right ventricular enlargement and dysfunction, respectively, and moderate PAH. Elevated pulmonary arterial pressure was substantially reduced and exercise capacity increased. Improvement was maintained over 2 years, and bosentan treatment continues in this patient. Our report is in line with a series of current reports in PPHT that support the use of bosentan in this subset of PAH patients.
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PMID:Successful treatment of portopulmonary hypertension with bosentan: case report. 1691 13

Pulmonary arterial hypertension (PAH) with coexisting portal hypertension has been defined as portopulmonary hypertension (PPHTN). It is often related to liver cirrhosis of various aetiologies and is associated with a high mortality rate. Endothelin-1 (ET) is supposed to play an important role in the pathogenesis of PAH as well as portal hypertension. Therefore, therapy with an ET(A)/ET(B) receptor antagonist might be of use in the treatment of PPHTN. We report the case of a 76-year-old male with liver cirrhosis owing to chronic hepatitis C virus infection and PPHTN who was treated with the dual ET(A)/ET(B) receptor antagonist bosentan. The patient showed remarkable improvement of 6-min walking distance from 300 to 480 m after 2 weeks and to 540 m after 14 weeks, respectively. In addition, a significant decline of N-terminal pro B-type natriuretic peptide fraction (NT-proBNP) from 4928 ng mL(-1) to 640 ng mL(-1) was observed. Bosentan might be a promising new therapeutical option for patients suffering from PPHTN.
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PMID:Bosentan treatment of portopulmonary hypertension related to liver cirrhosis owing to hepatitis C. 1691 14

Liver cirrhosis is a complex and progressive disease associated with high mortality. In developing countries, alcoholic liver disease is the most common form of liver cirrhosis, followed by chronic viral disease, especially hepatitis C virus infection. Cirrhosis is associated with systemic and splanchnic hemodynamic abnormalities, including increased vascular volume, decreased systemic vascular resistance, and increased cardiac output. At the splanchnic vascular bed, increases in portal flow and intrahepatic resistance have been described, inducing portal hypertension. Pulmonary arterial hypertension is a progressive disease of pulmonary circulation, without left ventricle and valvular heart disease; it is closely related with structural changes in pulmonary arteries. Idiopathic pulmonary arterial hypertension is related to abnormalities in cellular signals, inducing arterial hypertrophy and increased vascular tone. Porto-pulmonary hypertension includes simultaneous portal and pulmonary arterial hypertension. To confirm disease, it is important to exclude concomitant heart disease. Porto-pulmonary hypertension requires important components: portal hypertension, shear vascular stress, and cellular activation with pulmonary arterial hypertrophy. In this short manuscript, the epidemiology, patho-physiology, and diagnostic criteria of the disease are reviewed to optimize early diagnosis and treatment.
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PMID:[Epidemiology, physiopathology and diagnosis of pulmonary hypertension in hepatic (PH) cirrhosis]. 2262 15

Pulmonary arterial hypertension (PAH) that occurs in the setting of cirrhosis and portal hypertension is referred to as portopulmonary hypertension (PPHTN). Liver transplantation (LTx) is curative, but the presence of moderate-to-severe PPHTN may be a contraindication for transplantation because of the elevated risk of peri- and post-transplantation morbidity and mortality. We report a successful liver transplantation in a patient with liver cirrhosis after treatment of moderate-to-severe PPHTN with a combination of the dual endothelin receptor antagonist bosentan and the specific phosphodiesterase-5 inhibitor sildenafil.
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PMID:Treatment with a combination of bosentan and sildenafil allows for successful liver transplantation in a patient with portopulmonary hypertension. 2525 28