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)

Severe hypoxemia may occur in patients with liver disease as a result of abnormal intrapulmonary vasodilatations (hepatopulmonary syndrome, HPS). Liver transplantation (LT) is the only effective treatment of HPS, with a quite variable delay of improvement of oxygenation. Smoking, by decreasing respiratory nitric oxide (NO), apparently contributed to improved oxygenation in a 44-year-old man with alcohol-induced cirrhosis, complicated by HPS, who underwent LT. The patient quit smoking just before LT, when his PaO(2) was 29 mm Hg and exhaled NO (eNO) 28 ppb, a value far above the normal limits (9.6 +/- 3.2 ppb). After LT, oxygenation remained poor and eNO remained high for more than 4 months, when the patient started to smoke again (blood HbCO going up to 5%). At that time eNO decreased to 6 ppb and PaO(2) increased to 67 mm Hg. The strict relationship between eNO and oxygenation observed in this case reinforces the hypothesis that NO is the most important vasodilating mediator in HPS. Smoking may have hastened the resolution of HPS after LT by inhibiting respiratory NO and/or through a generalized impairment of endothelium-dependent vasodilation.
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PMID:Smoking and hypoxemia caused by hepatopulmonary syndrome before and after liver transplantation. 1148 30

The hepatopulmonary syndrom occurs when pulmonary microvacular dilatation causes hypoxemia in cirrhosis. It is found in between 15-20% of patients with chronic liver diseases and should be considered in the differential diagnosis of dyspnea or abnormal arterial oxygenation in this group. The presence of HPS appears to significantly increase mortality in affected patients with cirrhosis. The mediators of intrapulmonary vasodilatation and HPS are not fully characterized although pulmonary nitric oxide overproduction appears to be a key event in human and experimental models. Contrast echocardiography is the best screening test for pulmonary vasodilatation. Currently there are no effective medical therapies for HPS, although liver transplantation results in reversal of HPS in most cases. However, mortality is higher in patients with HPS undergoing transplantation relative to those without HPS.
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PMID:Hepatopulmonary syndrome. 1548 33

The objective of the study is to determine the diagnostic role of pulmonary functional tests and perfusion pulmonary scintigraphy for quantifying the oxygenation and vascular abnormality in patients with liver cirrhosis. The prospective study included 70 patients with liver cirrhosis. Arterial blood gases analysis were performed in both supine and sitting positions while inhaling room air, and 15 minutes after exposure of hyperoxic mixture. Perfusion pulmonary scintigraphy using albumin macroagregate labelled with radioactive technetium (99mTc-MAA) was performed for the visualisation of intrapulmonary vascular dilatation. The diagnosis of hepatopulmonary syndrome was made in 10 (14.3%) patients. The patients with hepatopulmonary syndrome had severe hypoxemia (Pa,O2 7.41 +/- 1.81 kPa), and poor response to 100% oxygen inhalation (Pa,O2 21.07 +/- 14.41 kPa) and higher alveolo-arterial gradient (5.73 +/- 2.65 kPa). Radioisotope marker 99mTc-MAA skipped intrapulmonary circulation in all patients with HPS and in no one without pulmonary vascular dilatations. The combined approach of 100% inspired oxygen and perfusion pulmonary scintigraphy may identify early oxygenation disorders and alter the priority for liver transplantation, especially in view of potential syndrome resolution.
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PMID:The significance of hepatopulmonary syndrome in liver transplantation. 1601 69

Hepatopulmonary syndrome (HSP) is characterized by the triad of advanced liver disease, arterial hypoxemia, and intrapulmonary vascular dilatation. Most cases of HSP are associated with cirrhotic portal hypertension; however, it has also been reported in acute liver failure patients. An estimated prevalence of HPS in patients with chronic liver disease is around 5-32%. Sarcoidosis is a granulomatous disorder with noncaseating granulomas involving the lungs in 90% of cases. Sarcoidosis can involve the liver but in only about 1% of cases does it lead to cirrhosis and portal hypertension. In this review, we report a rare case of liver cirrhosis due to sarcoidosis with associated hepatopulmonary syndrome and discuss this in detail.
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PMID:Hepatopulmonary syndrome and liver sarcoidosis: a tale of two diseases. 2001 47

Pulmonary vascular complications of liver disease comprise two distinct clinical entities: hepatopulmonary syndrome (HPS-microvascular dilatation and angiogenesis) and portopulmonary hypertension (POPH-vasoconstriction and remodeling in resistance vessels). These complications occur in similar pathophysiologic environments and may share pathogenic mechanisms. HPS is found in 15% to 30% of patients with cirrhosis and its presence increases mortality and the risks of liver transplantation, particularly when hypoxemia is present. Contrast echocardiography and arterial blood gas analysis are required to establish the diagnosis. No medical therapies are available, although liver transplantation is effective in reversing the syndrome. POPH is found in 4% to 8% of patients undergoing liver transplantation evaluation, and the presence of moderate to severe disease significantly increases perioperative transplant mortality. Transthoracic echocardiography is recommended for screening and right-heart catheterization is required to establish the diagnosis. Medical therapies are increasingly effective in improving pulmonary vascular hemodynamics in POPH and may result in better perioperative outcomes.
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PMID:Pulmonary complications of cirrhosis. 2112 31