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

Twenty patients with biopsy proved liver disease, and roentgenologic features of hypertrophic osteoarthropathy have been studied, and the literature has been reviewed. The syndrome is a rare association of many chronic liver diseases, including primary biliary cirrhosis, bile duct carcinoma, benign bile duct stricture, chronic active hepatitis, posthepatitic cirrhosis and alcoholic cirrhosis. Patients may be asymptomatic, although bone pain, arthralgia or arthritis may be presenting symptoms. Ninety per cent of the patients are clinical jaundiced at the time of diagnosis, and 95 per cent have digital clubbing. The distal tibia and fibula are the first bones to become involved, although wrist, foot bones, femurs, hand bones and humeri may be affected in order of frequency. There is no correlation between the presence of esophageal varices or surgical portacaval shunts and the extent of the syndrome, neither is there a correlation with the degree of liver function impairment. Serum calcium and phosphate levels are normal, as is urinary hydroxyproline and estrogen excretion. There was no evidence to implicate elevated levels of growth hormone or overdosage of vitamin A. Although the majority of patients tested had mild arterial hypoxemia, increased cardiac output and evidence of right to left shunting, these were also present in disease-matched control subjects without osteoarthropathy. For screening purposes, patients with chronic liver disease and clubbing should have roentgenologic studies of the lower tibias and fibulas, to select those patients suitable for a more extensive skeletal survey.
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PMID:Hypertrophic hepatic osteoarthropathy. Clinical, roentgenologic, biochemical, hormonal and cardiorespiratory studies, and review of the literature. 46 21

A study of the clinical and aetiological patterns of finger clubbing and hypertrophic osteoarthropathy was carried out over a 15-year period. 116 patients were studied. Pain is not a common symptom in patients with finger clubbing and osteoarthropathy in Nigerians, contrary to what has been reported in the literature. The cause of finger clubbing is predominantly pulmonary in origin, being responsible in 84 per cent of cases. The commonest cause in bronchiectasis, followed by empyema thoracis, bronchial carcinoma and lung abscess. Among the nonpulmonary causes are infective endocarditis, endomyocardial fibrosis and cirrhosis of liver. Hypertrophic osteoarthropathy is found in 15 cent of the patients with finger clubbing, the commonest cause being carcinoma of the bronchus.
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PMID:The clinical and aetiological pattern of finger clubbing and hypertrophic osteoarthropathy in Nigerians. 50 49

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

Between July 1986 and April 1989, 334 hospitalized adult Ethiopian patients with chronic liver disease were studied according to a protocol to define their clinical features and to identify risk factors with the aim of preventive intervention. Of these, 14 had chronic hepatitis, 208 cirrhosis and 112 hepatocellular carcinoma (HCC). Both clinical and histological diagnostic criteria were employed. A detailed questionnaire was used to document demographic and clinical data. A common clinical presentation among patients with chronic hepatitis was darkening of the face and hands with or without hypertrichosis of the face and blisters over the dorsi of the hands. This overt or latent form of porphyrea cutanea tarda (PCT) responds to chloroquine. Patients with cirrhosis of the liver commonly present for the first time with ascites, splenomegaly, haematemesis and/or melena from oesophageal varices, and mental changes due to hepatic encephalopathy. Overt or latent forms of PCT are also common features. Peculiar to these cirrhotics is the rarity of spider naevi, gynaecomastia, testicular atrophy, Dupuytren's contracture, parotid gland enlargement and clubbing of the fingers. Exhaustion, loss of appetite, rapid loss of weight, right upper quadrant and/or epigastric pain (all often of less than 6 months' duration, a big, hard, tender and grossly nodular liver with bruit, signs of portal hypertension, and/or hepatic encephalopathy, in a young male with a rapid down hill course characterize the Ethiopian patient with HCC. Serum anti-nuclear factor, anti-mitochondrial anti-bodies and anti-smooth muscle anti-bodies were absent in those with chronic hepatitis and were uncommon in the cirrhotics and HCC cases. One or more hepatitis B virus markers were found in 86% of chronic hepatitis, 88% cirrhosis and 78% HCC and the HBsAg carrier state was found in 36%, 29% and 23%, respectively. Among the HBsAg carriers, HBeAg positivity was less common than anti-HBe but anti-HDV was significantly higher than in the healthy general population. Alphafetoprotein (AFP) levels greater than 500 mg/ml were present in 16 (8%) cirrhotics and 58 (52%) patients with HCC. Histologically, 3 of the chronic hepatitis patients had progressed to cirrhosis, 8 of the cirrhotic patients had chronic active hepatitis and 85% of HCC cases occurred in a background of macronodular cirrhosis. Three cirrhotics developed HCC during follow-up.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Chronic liver disease in Ethiopia: a clinical study with emphasis on identifying common causes. 131

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

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

Musculoskeletal disease occurs in association with inflammatory bowel disorders including Crohn's disease and ulcerative colitis, as well as with Whipple's disease; with enteritis caused by Salmonella, Shigella, and Yersinia; and also following intestinal bypass surgery. Extraintestinal causes of musculoskeletal alterations include Laennec's and biliary cirrhosis and pancreatitis. Three types of musculoskeletal abnormalities are recognized in patients with inflammatory bowel diseases: peripheral joint arthritis, sacroiliitis and spondylitis identical to ankylosing spondylitis, and rarely, miscellaneous changes such as digital clubbing and hypertrophic osteoarthropathy.
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PMID:Enteropathic arthropathies. 243 70

Hypertrophic osteoarthropathy (HOA) may be an idiopathic condition or may be secondary to other diseases, the most common of which is bronchogenic carcinoma. Among non neoplastic etiologies, it is commonly associated with chronic liver disease, usually cirrhosis and chronic active hepatitis. The concomitant occurrence of HOA and hepatic steatosis is another association that has recently been reported. We report here a 70-year-old male with periostitis, clubbing of the fingers and alcoholic hepatitis stenosis. We emphasize the need to perform observational studies to validate this association.
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PMID:[Hypertrophic osteopathy and acropachy associated with noncirrhotic alcoholic liver disease. Apropos a case]. 249 73


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