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

Hypoxemia is a frequent occurrence in patients with severe hepatic disease. Multiple mechanisms have been implicated in the production of such hypoxemia. The case of a 35-year-old man with cyanosis, clubbing, and cirrhosis is presented. Physiologic data from this patient revealed normal pulmonary function, except for a low diffusing capacity and a 28 percent shunt while the patient was breathing 100 percent oxygen. A perfusion scan with radioactive 99mtechnetium-labelled macroaggregated albumin revealed 67 percent of the labelled macroaggregated albumin in the systemic circulation. Post-mortem examination demonstrated normal pulmonary parenchyma, markedly dilated intraparenchymal capillaries and arterioles, subpleural angiomata, and cirrhosis. No anatomic arteriovenous connections were demonstrated before or after death. We conclude that the arterial hypoxemia of some patients with hepatic cirrhosis results from dilated gas-exchanging blood vessels. These widened vessels prevent end pulmonary capillary oxygen tension from reaching equilibrium with alveolar gas, perhaps because of the widened distance for diffusion.
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PMID:Alveolar-capillary oxygen disequilibrium in hepatic cirrhosis. 63 Sep 68

This is the definitive review of the cardiovascular effects of liver disease. Physiologic and pathologic studies accomplished primarily in the 1950s and 1960s have been applied to a case of a 16-year-old boy with cirrhosis of the liver who presented with weakness, dyspnea, cyanosis, and clubbing. The probable mechanisms for his signs and symptoms are discussed in detail. The cause for the hyperkinetic circulation is unknown. By exclusion, the cyanosis is attributed to intrapulmonary shunting. Portapulmonary shunts are not quantitatively important. The suitability of the terms micronodular and macronodular cirrhosis is highlighted. Indications for various types of surgical portal shunts are discussed. The value of preoperative hemodynamic measurements of the portal circulation to the individual patient is debated. A liver transplant is considered in this case with no promising medical therapy and a very poor prognosis. The renal disease manifested by red cell casts in the urine is thought to be caused by an immunologic reaction in the kidney somehow related to his liver disease. This discussion is led by Dr. Jack Myers, but his opinions are correlated with those of a pathologist, radiologist, surgeon, and gastroenterologist. It is a clinical tour de force, although not all the predictions are confirmed by laboratory studies such as this patient's hepatic wedge pressure, pulmonary artery pressure, and cardiac fluoroscopy.
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PMID:Multidisciplinary Conferences in Gastroenterology. Cardiovascular effects of severe liver disease. 127 11

During the last 26 years, 21 patients with cirrhosis of the liver with severe cyanosis and gross clubbing simulating congenital cyanotic heart disease were subjected to cardiac catheterisation and angiography, splenography, liver function tests and liver biopsy. One patient had tetralogy of Fallot in addition, which was satisfactorily corrected. He continued to be intensely cyanosed and dyspnoeic despite adequate surgical correction of his cardiac defect as demonstrated on cardiac catheterisation and angiocardiography. Portupulmonary fistulas could not be demonstrated in any patient. The cyanosis and clubbing were secondary to right to left intrapulmonary shunting across multiple tiny pulmonary arteriovenous fistulas in all cases. In 16 cases selective pulmonary angiography revealed discrete arteriovenous fistulas. In 5 cases the angiogram did not reveal any convincing evidence of pulmonary arteriovenous fistulas. In 2 of these 5 cases peripheral vein contrast echocardiography, which seems to be a sensitive investigation, demonstrated right to left intrapulmonary shunting. Open lung biopsy in one case showed evidence of pulmonary arteriovenous fistulas.
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PMID:Cirrhosis of liver simulating congenital cyanotic heart disease. 193 51

Six cases of cyanosis and digital clubbing caused by liver cirrhosis were reported. The mean levels of PaO2 and PaCO2 were 6.4 +/- 0.8 kPa (48.3 +/- 6.2 mmHg) and 3.9 +/- 0.8 kPa (29.5 +/- 6.1 mmHg) respectively. The cause of cyanosis and digital clubbing in liver cirrhosis was discussed. Abnormal intrapulmonary shunting (IPS), which was proved in 4 cases by whole-body radionuclide scanning with 99mTc-MAA, is suggested as the major cause of cyanosis in liver cirrhosis. Clinically IPS is usually associated with debilitating conditions characterized by hyperventilation, abnormality of lung diffusion and orthodeoxia or platypnea. The authors suggest that whole-body radionuclide scanning with 99mTc-MAA may be performed for establishing IPS if liver cirrhosis is highly suspected to be the cause of severe hypoxemia.
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PMID:[Diagnosis of cyanosis and digital clubbing caused by liver cirrhosis]. 220 37

41 year-old male with liver cirrhosis accompanying severe hypoxemia was presented. Shortly after the diagnosis of liver cirrhosis, he suffered from exertional dyspnea and cyanosis. Though home oxygen therapy had been prescribed for 2 years, hypoxemia gradually progressed accompanied by persistent cough, mucous sputa and intermittent fever. The chest X-ray revealed bilateral interstitial shadow particularly localized in lower lung fields. The arteriovenous shunt ratio was shown to be 24% by oxygen method. Perfusion lung scan using 99mTc-labeled MAA revealed perfusion defects in bilateral lung fields and radionuclide uptake was strongly demonstrated in the kidneys. These clinical data suggested that severe hypoxemia was probably due to multiple arteriovenous shunt. With further progression of hypoxemia for 4 months, he died of hepatic failure and pulmonary infection. Autopsy showed Miyake's type B cirrhosis. Multiple pleural and subpleural arteriolar nevi were demonstrated grossly and microscopically. There were no arteriovenous malformations demonstrated after injection of barium-gelatin solution into the pulmonary artery. Histologically, irregularly dilated vessels were found in the lung parenchyma beneath the pleura and filled with blood and injection material. These clinical and pathological findings provided evidence that the mechanism of arterial desaturation was pulmonary arteriovenous shunting due to liver cirrhosis.
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PMID:[Hypoxemia of liver cirrhosis--an autopsy case study]. 229 Feb 37

A 12-year-old boy with Wilson's disease developed exertional dyspnea, cyanosis, and finger clubbing 10 months after diagnosis. The hypoxemia was caused by arteriovenous shunting, demonstrated by radionuclide scanning and pulmonary arteriography. Orthotopic liver transplantation was performed after the development of severe hypoxemia. There was no apparent reversal of the intrapulmonary arteriovenous shunting and he died 10 days posttransplantation of multiple organ failure secondary to hypoxemia. Monitoring arterial oxygen saturation in children with cirrhosis is warranted since the presence of significant arteriovenous shunting may influence prognosis and decisions regarding liver transplantation.
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PMID:Failure of liver transplantation in Wilson's disease with pulmonary arteriovenous shunting. 230 74

Pulmonary perfusion imaging with Tc-99m MAA revealed significant uptake in the lungs, brain, spleen, and both kidneys of a 48-year-old woman with liver cirrhosis and pulmonary telangiectasia associated with marked hypoxemia and cyanosis. Dynamic pulmonary perfusion imaging revealed a gradual reduction after peak uptake in both lungs. Several weeks after albumin replacement, the hypoxia and dyspnea disappeared with no change in hepatocellular function. At that time, dynamic pulmonary perfusion imaging revealed a plateau-like time-activity curve of uptake in the lungs, as compared with the findings obtained during the state of severe hypoxemia. These observations suggest that pulmonary telangiectasia in a patient with liver cirrhosis may be due to functional vasodilatation. Serial dynamic pulmonary perfusion imaging indicates the passage of the MAA particles through the widened lumen of the pulmonary alveolar capillaries.
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PMID:Reversibility of pulmonary telangiectasia in liver cirrhosis evidenced by serial dynamic pulmonary perfusion imaging. 260 48

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

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


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