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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Hepatopulmonary syndrome has yet not been sufficiently assessed in noncirrhotic portal hypertension. The prevalence of hepatopulmonary syndrome was determined in 31 consecutive patients with noncirrhotic portal hypertension (19 idiopathic portal hypertension, 7 portal vein thrombosis, 5 congenital hepatic fibrosis) and 46 patients with liver cirrhosis. Contrast echocardiography was carried out in all patients. Macroaggregated albumin lung perfusion scans were performed in patients with positive contrast echocardiogram. Hepatopulmonary syndrome was detected in 5 (10.8%) cirrhotic and 3 (9.7%) noncirrhotic portal hypertensive patients (2 idiopathic portal hypertension, 1 portal vein thrombosis). All patients with hepatopulmonary syndrome had an increased shunt fraction (13-62%) and a decreased diffusion capacity of carbon monoxide (40-79%), and 7 of them were hypoxemic (PaO2, 31.6-69.8 mm Hg). These findings show that hepatopulmonary syndrome may occur in both liver cirrhosis and noncirrhotic portal hypertension and that portal hypertension is the predominant etiopathogenic factor related to hepatopulmonary syndrome.
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PMID:Hepatopulmonary syndrome in noncirrhotic portal hypertensive patients. 1275 70

Hepatopulmonary syndrome--a complication of chronic liver disease-is characterised by hypoxaemia, which results from widespread intrapulmonary vascular dilatations. Amplified production of pulmonary nitric oxide is thought to be important in development of this disorder in patients with liver cirrhosis. Here, we report a 64-year-old man with hepatopulmonary syndrome associated with hepatitis-C-virus-related cirrhosis. We gave the patient nebulised N(G)-nitro-L-arginine methyl ester (L-NAME), an inhibitor of NO synthesis, which enhanced oxygenation (arterial oxygen pressure increased from 6.98 to 9.45 kPa). After L-NAME, the distance the patient could walk in 6 min rose by 92 m. Administration of L-NAME by aerosol might offer a new approach to treatment of hepatopulmonary syndrome.
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PMID:Effect on dyspnoea and hypoxaemia of inhaled N(G)-nitro-L-arginine methyl ester in hepatopulmonary syndrome. 1285

The majority of cirrhotic patients present dyspnea, but most of the time this is a consequence of ascites, hepatic hydrothorax or cardiopulmonary disease. Very few of these patients undergo the hepatopulmonary syndrome, an infrequent complication of chronic liver disease, especially when associated with portal hypertension and cirrhosis. Hepatopulmonary syndrome is characterised by dyspnea and, consequently, arterial hypoxemia, due to intrapulmonary vasodilation and shunting. The real pathogenesis of this vasodilation is still unknown, although many hypotheses have been suggested. Furthermore, no medical therapy appears to be really useful in the treatment of hepatopulmonary syndrome. At the present time, the only treatment that is efficacious for the resolution, or for the improvement of blood gas oxygenation abnormalities, is orthotopic liver transplantation.
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PMID:Hepatopulmonary syndrome. A review of the literature. 1285 33

Inhibition of tumour necrosis factor-alpha (TNF-alpha), levels of which are increased in the blood of cirrhotic rats, prevents hyperdynamic circulatory state, mainly by decreasing the vascular overproduction of nitric oxide. Hepatopulmonary syndrome, which is characterised by intrapulmonary vascular dilatation and increased alveolar to arterial oxygen tension difference (PA-a,O2), is mainly related to pulmonary over-production of NO by macrophages accumulated in lung vessels. Since TNF-alpha is a potent activator of macrophagic inducible nitric oxide synthase (NOS), the aim of this study was to investigate whether TNF-alpha inhibition prevented hepatopulmonary syndrome and hyperdynamic circulatory state in rats with cirrhosis. TNF-alpha was inhibited by 5 weeks of pentoxifylline (10 mg x kg body weigh(-1) x day(-1)) in rats with cirrhosis induced by common bile duct ligation. Cardiac output, pulmonary and systemic vascular resistance, PA-a,O2 and cerebral uptake of intravenous technetium-99m-labelled albumin macroaggregates (which reflects intrapulmonary vascular dilatation) were similar in sham- and pentoxifylline-treated cirrhotic rats. Blood TNF-alpha concentrations and pulmonary intravascular macrophage sequestration, as assessed by morphometric analysis and radioactive colloid uptake, were decreased with pentoxifylline. Pentoxifylline also prevented increases in aorta and lung NOS activities and inducible NOS expression. Thus pentoxifylline prevents development of hyperdynamic circulatory state and hepatopulmonary syndrome, probably by inhibiting the effects of tumour necrosis factor-alpha on vascular nitric oxide synthase and intravascular macrophages. These results support an important role for tumour necrosis factor-alpha in the genesis of hepatopulmonary syndrome.
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PMID:Prevention of hepatopulmonary syndrome and hyperdynamic state by pentoxifylline in cirrhotic rats. 1517 75

Nonalcoholic fatty liver disease (NAFLD) has been reported in adults with hypothalamic or pituitary dysfunction, and some have progressed to end stage liver disease requiring transplantation. We report a teenager who initially presented with hypoxia due to intrapulmonary shunting, found to have NAFLD and cirrhosis associated with a hypothalamic tumor and panhypopituitarism. The NAFLD recurred very quickly after a successful liver transplant. Hepatopulmonary syndrome may be more common in cirrhosis associated with pituitary dysfunction than in other types of cirrhosis.
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PMID:Rapid recurrence of nonalcoholic fatty liver disease after transplantation in a child with hypopituitarism and hepatopulmonary syndrome. 1569 May 45

Hepatopulmonary syndrome (HPS) is a well-known complication characterized by abnormalities of arterial oxygenation in patients with chronic liver disease, with or without portal hypertension. There is no definitive treatment for HPS except liver transplantation. We report on a patient with HBV-related liver cirrhosis and HPS who received garlic and lamivudine for 3 years. Signs of liver failure and hypoxemia gradually improved, indicating that lamivudine may improve the functional reserve of the liver, while garlic may help to reduce the signs and symptoms of HPS.
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PMID:Is there any medical therapeutic option in hepatopulmonary syndrome? A case report. 1583 81

Hepatopulmonary syndrome consists of a triad of chronic liver disease, hypoxemia, and intrapulmonary vascular dilatation in the absence of primary cardiac or pulmonary diseases. Though this complication has been described in as many as 4-29% of patients with liver disease, its clinical manifestations are often subclinical. The standard diagnostic tests for hepatopulmonary syndrome are the applications of contrast echocardiography and 99mTc lung perfusion scan. In this report, we describe a 19-year-old female with biliary atresia and subsequent liver cirrhosis, who also had the complication of hepatopulmonary syndrome since adolescence. We conclude when the symptoms and signs of hypoxemia appear in a patient with chronic liver disease, the differential diagnosis of hepatopulmonary syndrome should be kept in mind.
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PMID:Hepatopulmonary syndrome: report of one case. 1638 37

Hepatopulmonary syndrome (HPS) is charaterised by arterial hypoxaemia found mainly in association with chronic liver disease (most commonly cirrhosis of liver) but also rarely with acute liver diseases like fulminant hepatic failure or Budd-Chiari syndrome. The purpose of this article is to present an uncommon but grave complication mostly associated with cirrhosis of liver and to differentiate this entry from cyanotic heart diseases or certain lung conditions. Investigations include history and physical examination, certain biochemical tests, upper gastro-intestinal (GI) endoscopy, abdominal ultrasonography, liver biopsy and venacavography in appropriate cases. Finally, the suspected cases underwent arterial blood gas (ABG) analysis and contrast enhanced echocardiography (CEE) for confirmation of the diagnosis of HPS. Of the 123 cases of cirrhosis of liver, three cases of HPS (2.4%) were found-all of them being males. Also one male patient with inferior vena cava (IVC) obstruction amongst other causes presenting with HPS was encountered. As of now, no medical treatment has been proved to be useful and liver transplant remains the only hope for this disorder.
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PMID:Hepatopulmonory syndrome--an uncommon and severe complication of certain liver diseases. 1649 66

Hepatopulmonary syndrome (HPS) is a clinical state defined by a chronic hepatic disorder, intrapulmonary vascular dilatation, and altered gas exchange resulting in hypoxemia. Cirrhosis of the liver is the most common condition associated with HPS. A 3-year-old boy who presented with end-stage liver disease and severe hepatopulmonary syndrome underwent orthotopic liver transplantation (OLT). The findings of HPS resolved immediately after OLT. His status is within normal limits at 6 months after liver transplantation.
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PMID:Liver transplantation of a child with child a cirrhosis and severe hepatopulmonary syndrome. 1679 24

Hepatopulmonary syndrome consists of a clinical triad: arterial blood deoxygenation, intrapulmonary vasodilation, and liver disease. Both acute and chronic cases of this syndrome have been reported, and the most common cause is cirrhosis. The principle disease mechanism is dilation of the pulmonary blood vessels causing alterations in gas exchange. Increased pulmonary production of nitric acid has been implicated as the primary pathogenic mechanism of vasodilation although it has also been associated with imbalance between vasodilators and vasoconstrictors. We describe the case of a patient with hepatopulmonary syndrome and adenocarcinoma of the colon with metastases to a previously healthy liver.
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PMID:[Hepatopulmonary syndrome in a patient with adenocarcinoma of the colon metastatic to the liver and no apparent chronic liver disease]. 1704 Jun 63


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