Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Between October, 1976 and December, 1982, 176 patients with full-blown liver cirrhosis were referred to us either with Acute oesophageal variceal bleeding, or after having had such a haemorrhage. Eight of these patients died of a haemorrhage that did not respond to conservative treatment, or of hepatic failure after emergency surgery. After receiving initial conservative treatment, 168 patients were classified in accordance with clinical, biochemical and haemodynamic selection criteria, particular importance being attached to the haemodynamic criteria. One-hundred-and-one of these patients in whom a shunt operation was not justifiable, were treated conservatively by means of repeated sclerotherapy. In the cases of acute bleeding, a balloon tamponade was performed in 3, an emergency shunt procedure in 1 and palliative dissection surgery in 4. Twenty-three patients in a non-bleeding state were treated conservatively without sclerotherapy. Shunt surgery was performed in 44 patients; out of the 42 patients shown by our selection criteria to be suitable for shunt surgery, only 1 patient died, 5 days after the operation, of cardiac failure with underlying toxic cardiomyopathy. In view of the low early mortality rate in selectively operated patients, a major argument against the use of the shunt for the treatment of portal hypertension is no longer valid, and it should again be considered whether the surgical procedure might not provide better long-term results than does conservative sclerotherapy alone.
 ...
PMID:Stricter selection criteria improve the results of shunt surgery. 387 35

An autopsy case of hypertrophic obstructive cardiomyopathy with extensive myocardial fibrosis is reported in a 43-year-old male. His mother died suddenly at 55. At the age of 39 the patient felt fatigue and feverish sensation followed by dyspnea and palpitation on exertion. He responded to beta-blocker and was discharged on the 51st hospital day. He died suddenly during his work three years and one month after discharge. The heart weighs 700 g. The thickness of the ventricular septum measures up to 3.2 cm, and that of the left ventricular posterior wall 2.2 cm. Subaortic endocardium is moderately thickened. Many patchy fibroses of various sizes and broad linear fibroses are mainly observed in the ventricular septum and in the left ventricular free wall. Microscopic examination shows severe fascicular disarray of hypertrophied myocardial fibers in the ventricular septum and in a part of the left ventricular anterior wall. Pericardial fibrosis, granulation tissue with many capillaries, and slight lymphocytic infiltrate are also noted. These findings suggest that the patient have both congenital hypertrophic cardiomyopathy and myocarditis. There are following possibilities as regards the relation between the two: first, haphazard association of cardiomyopathy with myocarditis; secondly, myocarditis triggered the onset or progression, or both, of cardiomyopathy. He also had liver cirrhosis, probably alcoholic, which appears to accelerate the progression of myocardial disarray and fibrosis.
 ...
PMID:[Hypertrophic obstructive cardiomyopathy with extensive myocardial fibrosis: case report with autopsy]. 403

We analyzed survival and causes of death among 163 patients with primary hemochromatosis diagnosed between 1959 and 1983. The mean follow-up period was 10.5 +/- 5.6 years (+/- S.D.). Cumulative survival was 92 per cent at 5 years, 76 per cent at 10 years, 59 per cent at 15 years, and 49 per cent at 20 years. Life expectancy was reduced in patients with cirrhosis of the liver as compared with those without cirrhosis (P less than or equal to 0.05), in patients with diabetes mellitus as compared with those without diabetes (P less than or equal to 0.002), and in patients who could not be depleted of iron during the first 18 months of venesection therapy as compared with those who could be depleted (P less than or equal to 0.001). Prognosis was not influenced by sex (P less than or equal to 0.5). Patients without cirrhosis had a life expectancy that was not different from that expected in an age- and sex-matched normal population. Analysis of the causes of death in 53 patients, as compared with the normal population, showed that liver cancer was 219 times more frequent among the patients (16 patients), cardiomyopathy was 306 times more frequent (3 patients), liver cirrhosis was 13 times more frequent (10 patients), and diabetes mellitus was 7 times more frequent (3 patients). Death rates for other causes, including extrahepatic carcinomas (seven patients), were not different from the rates expected. We conclude that patients with hemochromatosis diagnosed in the precirrhotic stage and treated by venesection have a normal life expectancy, whereas cirrhotic patients have a shortened life expectancy and a high risk of liver cancer even when complete iron depletion has been achieved.
 ...
PMID:Survival and causes of death in cirrhotic and in noncirrhotic patients with primary hemochromatosis. 405 6

Cardiomyopathy in alcoholics is considered to be associated with a low incidence of hepatic cirrhosis. To evaluate cardiac hemodynamics in alcoholic liver disease, left ventricular function in 37 patients with hepatic cirrhosis (group II) was compared with that in 13 normal subjects (group I) matched for age, sex and cardiac size. These groups were contrasted with group III, comprising 32 alcoholics without cirrhosis who had cardiac symptoms but no cardiomegaly or heart failure. Patients with cirrhosis as a group did not differ from normal subjects (group I) in terms of left ventricular filling pressure and cardiac muscle and pump function (cardiac index). However, subgroup IIA (n = 21) had a stroke index significantly less than normal, while subgroup IIB had a significantly increased stroke index and myocardial cardial contractility with a diminished systemic arterial resistance. Similar hepatic abnormalities were present in both subgroups. In group III, left ventricular end-diastolic and aortic mean pressures were significantly elevated compared with values in normal subjects, while cardiac index and indexes of ventricular contraction and relaxation were abnormal. Further examination of patients with cirrhosis indicated that the responses to volume or pressure increments in terms of the level of stroke work for a given filling pressure were most abnormal in group IIA, approximating those of group III. Thus, although overt cardiomyopathy is infrequent in patients with cirrhosis, asymptomatic myocardial disease may assume clinical importance during volume or pressure overload.
 ...
PMID:Cardiac function in alcoholics with cirrhosis: absence of overt cardiomyopathy--myth or fact? 669 42

Though myocardial alterations are well recognized in haemochromatosis, little attention has been paid to the cardiac changes in Wilson's disease. To define the extent of myocardial degeneration in newly diagnosed or chronically treated Wilson's disease, we reviewed the autopsy findings in 9 cases with this condition. We compared our observations with those in 3 control cases, selected for comparable age and with liver disease having no known association with cardiac degeneration. Our results revealed cardiac hypertrophy in 5 out of 9 cases of Wilson's disease. There was evidence of interstitial and replacement fibrosis, intramyocardial small vessel sclerosis and focal inflammatory cell inflammation to a variable degree in all cases. One case had AV nodal degeneration, and a 15 year old boy had severe atherosclerosis of the left main coronary artery. Two patients died suddenly, presumably secondary to an arrhythmia; one of these patients had the most marked myocardial alterations. We could not correlate these changes specifically with the tissue levels of copper, treatment with D-penicillamine, or the presence of cirrhosis. We conclude that there are definite morphological abnormalities in the hearts of patients with Wilson's disease consistent with a cardiomyopathy. Though the myocardial changes were non-specific, the fact that 2 patients died suddenly, suggests the need for a prospective study of cardiac function in these patients in the future.
 ...
PMID:The cardiomyopathy of Wilson's disease. Myocardial alterations in nine cases. 715 67

The major clinical problem in patients with thalassemia is iron overloading usually resulting from increased exogenous iron absorption from transfusions. Diseases of various organ systems result, including cirrhosis, cardiomyopathy, diabetes, and other less well appreciated endocrinopathies. Since 1976, we have routinely studied these patients with abdominal computed tomography (CT) and also have scanned other areas of clinical interest. It is the purpose of this report to examine the findings in 35 patients with severe beta-thalassemia and associated hemochromatosis in whom we have tabulated the pertinent CT, clinical and laboratory data.
 ...
PMID:Computed tomographic analysis of beta-thalassemic syndromes with hemochromatosis: pathologic findings with clinical and laboratory correlations. 736 13

We have previously demonstrated that impairment of cardiac contractility in a rat model of cirrhosis may be secondary to altered cardiac plasma membrane physical properties affecting beta-adrenergic receptor function. It is unclear whether this is caused by the cirrhosis or by the portal hypertension in this model, so we studied cardiomyocyte plasma membrane physical properties, lipid composition, and beta-adrenergic receptor function in a rat model of prehepatic portal hypertension without cirrhosis. Portal hypertension was induced by graded portal vein stenosis, whereas controls had a sham operation. Cardiac sarcolemmal plasma membrane physical properties were studied by fluorescent polarization methods using diphenylhexatriene and a series of anthroyloxy-stearic acids to estimate, respectively, the static and dynamic components of membrane fluidity. beta-Adrenergic receptor density and binding affinity were measured using 3H-dihydroalprenolol as a radioligand. Isoproterenol-stimulated adenylyl cyclase activity was measured by radioimmunoassay. Neither the static nor the dynamic components of membrane fluidity were significantly different in portal-hypertensive rats compared with the controls. The membrane cholesterol, phospholipid content, and the cholesterol/phospholipid ratio did not differ between the two groups. beta-Adrenergic receptor density and binding affinity in the portal-hypertensive rats (respectively, 1.65 +/- 0.06 pmol/mg protein and 6.78 +/- 0.80 nmol/L) were not different from controls (1.76 +/- 0.17 pmol/mg protein and 7.25 +/- 1.48 nmol/L). Isoproterenol-stimulated adenylyl cyclase activity in membranes from portal-hypertensive rats did not significantly differ from controls. We conclude that cardiac plasma membrane physical properties and beta-adrenoceptor function in portal hypertensive rats remains unimpaired, suggesting that the cardiomyopathy of cirrhosis is not caused by portal hypertension per se.
 ...
PMID:Cardiac plasma membrane physical properties and beta-adrenergic receptor function are unaltered in portal-hypertensive rats. 760 11

Based on anecdotal impressions, there is a common clinical perception that alcoholics with liver disease do not develop cardiomyopathy and that those with alcohol-induced cardiac disease are spared cirrhosis. To determine the relationship between alcoholic cardiomyopathy and cirrhosis, we carried out a prospective cross-sectional study that included: (1) 30 alcoholic men with cardiomyopathy; (2) 30 alcoholic men without cardiomyopathy (left ventricular ejection fraction > 55%); (3) 20 actively drinking alcoholics with cirrhosis; (4) 15 abstaining alcoholics with cirrhosis; and (5) 15 nonalcoholics with cirrhosis of other etiologies. Cirrhosis was observed in 13 of 30 patients with alcoholic cardiomyopathy (43%), compared with 2 of 30 alcoholics without cardiomyopathy (6%) (P < .001). Ten of the 20 active alcoholics with cirrhosis (50%) showed evidence of dilated cardiomyopathy. Actively drinking alcoholics with cirrhosis had a significantly lower mean ejection fraction and shortening fraction, as well as a greater mean end-diastolic diameter and left ventricular mass than abstaining alcoholics with cirrhosis. Cardiac studies of patients with nonalcoholic cirrhosis were normal. We conclude that a positive correlation exists between alcoholic cardiomyopathy and cirrhosis. Alcoholics admitted solely for cardiomyopathy have a higher prevalence of cirrhosis than unselected alcoholics without heart disease. Actively drinking alcoholics admitted only for cirrhosis show impaired cardiac performance, whereas abstaining alcoholics with liver disease tend to manifest normal cardiac function.
 ...
PMID:Relationship between cardiomyopathy and liver disease in chronic alcoholism. 763 21

Haemochromatosis is one of the most common inborn errors of metabolism. In prospective epidemiological studies the frequency of haemochromatosis is 0.0037 (76/20333 subjects) for homozygotes which corresponds to a gene frequency of 0.061 and a frequency of heterozygotes of 0.115. Abnormality in liver function tests, weakness and lethargy, skin hyperpigmentation, diabetes mellitus, arthralgia, impotence and ECG abnormalities are the most frequent findings and symptoms at diagnosis. In recent years about 50% of patients were detected without having liver cirrhosis and 20% of patients did not have any symptoms and pathology except iron overload. Survival analyses in long-term studies showed that in the absence of cirrhosis and diabetes, iron removal by phlebotomy therapy prevents further tissue damage and guarantees a normal life expectancy. Patients with massive and long-lasting iron overload had a worse prognosis than those with less severe iron excess. Iron removal in general ameliorated liver disease, weakness and cardiac abnormalities, and also prevented the progression of endocrine alterations. Therapy, however, did not influence insulin-dependent diabetes. Most deaths in patients with hereditary haemochromatosis were caused by liver cancers which often occurred many years after complete iron removal. In patients with haemochromatosis, liver cirrhosis, cardiomyopathy, and diabetes mellitus are also significantly more frequent causes of deaths when compared with the general population. Further strategies have to evaluate the design of screening programmes in order to diagnose more patients in the precirrhotic and asymptomatic stage.
 ...
PMID:Clinical spectrum and management of haemochromatosis. 788 Nov 58

Long-term blood transfusions lead to the accumulation of iron that in the absence of chelation therapy causes complications such as liver cirrhosis, growth failure, hypogonadism, hypothyroidism, hypoparathyroidism, diabetes and myocardiopathy. The last still represents the most frequent cause of death in haemosiderotic transfusion-dependent patients. At the moment the only chelator widely used is desferrioxamine (DFX). The drug works best when administered as a continuous infusion, mainly by the subcutaneous route. To patients with severe iron overload, impending organ failure, or poor compliance to chelation, DFX can be administered intravenously, through an externalized central catheter or, preferably, a subcutaneous port. Several studies have shown the effectiveness of DFX in reducing the iron burden, thus preventing the complications, once considered inevitable, of iron overload, and even in reverting some, but not all, of the iron-induced dysfunctions. Practical and psychological support are necessary to ensure satisfactory compliance with a therapy that is cumbersome and difficult. Toxic effects of DFX such as growth failure, hearing impairment and bone abnormalities seem to occur mainly in patients who have received high doses of DFX despite a low iron burden. Visual loss and renal and pulmonary toxicities, on the contrary, seem to be more directly related to high DFX peak doses administered irrespective of the patient's amount of iron overload. After bone marrow transplantation, phlebotomy or erythrocytoapheresis might be necessary to reduce further the iron accumulated during years of transfusions.
 ...
PMID:Clinical manifestations and therapy of transfusional haemosiderosis. 788 Nov 60


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>