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)

During the last 25 years, 20 patients with cirrhosis of liver with severe cyanosis and gross clubbing simulating congenital cyanotic heart disease were subjected to cardiac catheterization and angiography, splenography, liver function tests, and liver biopsy. No portopulmonary fistulas could be demonstrated. The cyanosis and clubbing were secondary to right to left intrapulmonary shunting across multiple tiny pulmonary arteriovenous fistulas. In 15 cases, selective pulmonary angiography revealed discrete arteriovenous fistulas. In five cases, the angiogram did not reveal any convincing evidence of pulmonary arteriovenous fistulas. In two of these five cases, peripheral vein contrast echocardiography demonstrated right to left intrapulmonary shunting and seems a sensitive investigation. Open lung biopsy in one case showed evidence of pulmonary arteriovenous fistulas.
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PMID:Cirrhosis of the liver simulating congenital cyanotic heart disease. 279 83

The hypoxaemia associated with hepatic cirrhosis is classically attributed to an intrapulmonary shunt caused by small vascular abnormalities. Severe hypoxaemia (47 mmHg) associated with dyspnoea, cyanosis and clubbing was observed in a 57-year old man who presented with cirrhosis of the liver. At contrast echocardiography, a right-to-left shunt was demonstrated by the appearance of microcavities in the left atrium and ventricle after peripheral intravenous injection of the contrast medium. The intrapulmonary location of the shunt was determined by a 4 cardiac cycles interval between the arrival of the microcavities in the right heart and their appearance in the left heart. The right-to-left shunt was confirmed by the pure oxygen ventilation test and by pulmonary perfusion scintigraphy with radiolabelled albumin microaggregates. Pulmonary angiography proved normal. Thus, contrast echocardiography is capable of diagnosing right-to-left shunts associated with hepatic cirrhosis and to demonstrate their intrapulmonary location.
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PMID:[Intrapulmonary right-left shunt associated with liver cirrhosis: diagnosis by contrast echocardiography]. 313 12

Hypoxaemia and digital clubbing are rare but well recognised associations of hepatic cirrhosis with portal hypertension. We report the first European patient with idiopathic non-cirrhotic portal hypertension complicated by these features. Pulmonary physiological studies show the hypoxaemia to be the result of anatomical and physiological intrapulmonary shunting.
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PMID:Non-cirrhotic portal hypertension with hypoxaemia. 334 7

A group of 258 patients with various forms of alcoholic liver disease-steatosis, mild and severe hepatitis, and cirrhosis-has been studied. Severity of disease as judged histologically did not correlate very well with clinical presentation although signs of hepatocellular failure were certainly commoner in severe hepatitis and cirrhosis. Fever, pigmentation, and clubbing were also pointers to these two conditions. Alcoholic hepatitis is probably precirrhotic and carries a poor prognosis and the best laboratory indicators of this are moderate elevation of white cell count and bilirubin. Prognosis in alcoholic liver disease is significantly improved by abstinence from alcohol.
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PMID:Studies in alcoholic liver disease in Britain. I. Clinical and pathological patterns related to natural history. 436 73

There was no significant difference in forearm muscle blood flow, measured by the clearance of (133)Xenon when 38 patients with liver disease were compared with 38 normal subjects. Patients with a clinically hyperdynamic circulation, finger clubbing, and previous portocaval anastomoses were included in the study. The changes in forearm skeletal muscle blood flow and pulse rate caused by a head-up tilt of 70 degrees were measured in 15 patients with chronic liver disease and 15 age-matched controls. Head-up tilting resulted in significantly less peripheral vasoconstriction and tachycardia in the group with liver disease than in the control group. These results suggest an impairment of baroreceptor-mediated sympathetic reactivity in liver disease. Such a defect might explain the relative rarity of hypertension in patients with cirrhosis.
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PMID:Skeletal muscle blood flow and neurovascular reactivity in liver disease. 471 2

A study of 510 patients in Scotland and northeastern England with histological evidence of alcohol-induced liver disease showed no difference in the age of presentation between males and females. Single men and widowed females were particularly susceptible to alcoholic liver disease. The social class distribution was similar to the population in general. Women were more reluctant to volunteer a history of alcoholism than men, they had a higher incidence of previous psychiatric illness (usually due to alcohol abuse) and they developed liver disease at lower consumption thresholds of alcohol than men. Patients under 40 years of age were more likely to have alcoholic fatty liver and less likely to have active cirrhosis than those over 40. Most often, the presenting symptoms were non-specific and tended to be related to the gastrointestinal system, particularly in women. Five per cent of patients were asymptomatic and 14% came to hospital for conditions other than alcoholic liver disease. Important clues to asymptomatic alcoholic liver disease included hepatomegaly, clubbing of the fingers and abnormal liver function tests. Gastro-oesophageal varices accounted for 40% of instances of haemorrhage and the mortality from upper gastrointestinal bleeding was 17%. Anaemia was the most common haematological abnormality. Alcoholic hepatitis was observed more frequently in the Glasgow area then elsewhere.
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PMID:Alcoholic liver disease in Scotland and northeastern England: presenting features in 510 patients. 660 94

A 10.5 year old girl with liver cirrhosis due to AAT-deficiency (Pi type ZZ) developed cyanosis and clubbing of finger and toes. Clinical aspect of a cyanotic heart disease appeared with 10 years, 7 years after diagnosis of cirrhosis. By contrast echocardiography existence of intrapulmonary arterio-venous shunts was demonstrated. When determined during the first year of life, serum-alpha-1-globulin-fraction of the patient was found to be normal. The result indicates, that even in severe AAT-deficiency of Pi type ZZ direct determination of AAT is necessary for diagnosis of the disease.
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PMID:[Liver cirrhosis due in alpha-1-antitrypsinin deficiency and development of an arteriovenous shunts of the lungs]. 660 21

A prospective survey of radiological bone and joint changes was undertaken in 42 patients with primary biliary cirrhosis (PBC) and 23 patients with alcoholic or cyptogenic cirrhosis who formed a control population. PBC patients were commonly found to have hypertrophic osteoarthropathy (38%), joint erosions (31%) and osteoporosis, these results being significantly different from the control group. Hypertrophic osteoarthropathy most frequently affected the first metacarpal and was rarely associated with finger clubbing or clinical symptoms. Joint erosions were most evident in the hands, often associated with a positive rheumatoid factor but only accompanied by symptomatic arthritis in four cases. Patients with PBC show a high prevalence of symptomless bone and joint changes which may become manifest clinically only as the disease progresses; radiography of the hands is recommended as a screening test for these changes.
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PMID:A prospective survey of radiological bone and joint changes in primary biliary cirrhosis. 723 12

Chronic liver disease is well known to be associated with pulmonary abnormalities. Hypoxemia, clubbing, cyanosis and hyperventilation are common. The hypoxemia in cirrhotic patients has several causes: diffuse shunts due to intrapulmonary arteriolar vasodilatation, impaired hypoxic vasoconstriction, impaired matching of ventilation to perfusion, pleural effusions and diaphragmatic dysfunction. Because of gravity, shifting of blood to the dilated precapillary beds of the lung bases results in an increased hypoxemic dyspnea when the patient is in the upright position, also known as orthodeoxia and platypnea. It has only been described in 5% of the cirrhotic patients and has not been described in a Belgian refereed journal (Medline literature search 1983-Aug 1993). It should be considered in the initial differential diagnosis of hypoxemia in patients with liver cirrhosis and dyspnea. Measuring arterial blood gases in the lying and upright position can prevent further invasive investigations, and whole body nuclide scan with technetium-99m macroaggregated albumin can confirm the diagnosis. Standard therapy with spironolactone (Aldactone) can worsen the condition and we found no additional benefit of beta-antagonists (propranolol/Inderal) in the reduction of the shunt fraction, probably because the main reason for the shunting is precapillary vasodilatation. Since there are no anatomical porto-pulmonary shunts, surgery is also inappropriate. The only therapy consists of oxygen supplements and low dose diuretics in patients with edema.
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PMID:Orthodeoxia and platypnea in liver cirrhosis: effects of propranolol. 751 28

The ventilation-perfusion relationships of the lung (VA/Q) and central haemodynamics were studied in seven patients with cirrhosis before and 30 min after a bolus injection of a somatostatin analogue, octreotide (Sandostatin, 50 micrograms i.v.), to elucidate the role of this substance in the hepatopulmonary syndrome. In the basal state all patients had normal spirometry but reduced diffusing capacity. Three patients had various degrees of hypoxaemia (6.9-8.3 kPa) and three had clubbing of the fingers. In the basal state VA/Q distributions, determined by inert gas elimination technique, showed an intrapulmonary shunt of 7.9 +/- 2.2% of cardiac output (range 1.5 to 17.1) and perfusion of lung regions with "low VA/Q" of 4.4 +/- 2.2% of cardiac output (range 0 to 15.4). After octreotide, the amount of shunting increased (10.9 +/- 4.4% of cardiac output; non-significant), while "low VAQ" was unchanged (3.7 +/- 1.3% of cardiac output). Arterial oxygen tension decreased from 10.2 +/- 1.1 to 9.7 +/- 1.1 kPa (non-significant). The mean pulmonary arterial pressure increased from 14.5 +/- 1.9 to 16.3 +/- 1.8 mmHg (p < 0.01). No alterations were seen in heart rate, stroke volume, cardiac output, central pressures or vascular resistances. The results of the present study do not support the hypothesis that octreotide improves hypoxaemia and ventilation-perfusion relationships in patients with hepatopulmonary syndrome.
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PMID:Ventilation-perfusion relationships and central haemodynamics in patients with cirrhosis. Effects of a somatostatin analogue. 796 22


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