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)

During childhood, Wilson's disease becomes manifest mostly in the hepatic form. In children every case of cirrhosis of the liver, hemolysis with high levels of conjugated bilirubin in the serum, and otherwise in explicable tremor make it imperative to exclude or confirm the existence of Wilson's disease. A false diagnosis often delays the start of therapy with d-penicillamine and low-copper diet. The prognosis, which was still fatal a few years ago, has improved considerably thanks to new therapeutic possibilities.
 ...
PMID:[Morbus Wilson--pathogenesis, diagnosis, therapy, and course (author's transl)]. 60 Feb 71

We have studied the cytotoxicity against rabbit liver cells of lymphocytes from the peripheral blood of 71 patients with various liver diseases. The group with chronic active hepatitis and three patients with acute alcoholic hepatitis showed significantly higher mean values of lymphocytotoxicity (P less than 0.001) compared with the other patients with chronic persistent hepatitis, post-necrotic fibrosis and cirrhosis. Wilson's disease, and prolonged viral hepatitis. The mean cytotoxicity of these last groups did not differ significantly from controls. In four out of six patients with chronic active hepatitis a significant decrease of lymphocytotoxicity was found after immunosuppressive therapy with oral prednisolone. A good correlation between the lymphocytotoxicity test and histological signs of activity suggests that a cell-mediated immune aggression is present in this disease.
 ...
PMID:Lymphocytotoxicity test against rabbit hepatocytes in chronic liver diseases. 63 39

A normal exocrine pancreatic function was demonstrated by the secretin-pancreozymin-test in five patients with Wilson's disease either without (n = 2) or with cirrhosis of the liver but without portal hypertension (n = 3). In another patient with cirrhosis of the liver without portal hypertension the pancreas was normal at post mortem examination. In two patients with cirrhosis of the liver and portal hypertension bicarbonate (n = 1) and amylase secretion (n = 2) were diminished. The regression of portal hypertension under therapy with penicillamine in one of the latter cases was paralleled by the return to normal of exocrine pancreatic function. It is concluded that exocrine pancreatic insufficiency in Wilson's disease is dependent on the development and the progression of chirrhosis of the liver and not due to a primary manifestation of the disease itself.
 ...
PMID:[Involvement of the exocrine pancreas in Wilson's disease? (author's transl)]. 71 25

A patient with cryptogenic cirrhosis was found to have corneal pigmentation rings indistinguishable from Kayser-Fleischer rings on slit-lamp examination. Although she had hepatic encephalopathy that included confusion, tremor, and slurred speech, diagnosis of Wilson's disease was ruled out because urinary cooper excretion and hepatic copper concentrations were below the range found in symptomatic Wilson's disease. The exact nature of these rings could not be determined, and they were considered as Kayser-Fleischer-like rings.
 ...
PMID:Kayser-Fleischer-like ring in a cryptogenic cirrhosis. 71 54

Increased liver copper concentration and raised serum ceruloplasmin were demonstrated in primary biliary cirrhosis and disorders of the biliary tract, and occasionally in chronic active hepatitis and cirrhosis of the liver. Eight of 13 patients with primary biliary cirrhosis had liver copper content as high as seen in patients with hepatolenticular degeneration (is greater than 250 mjg/g dry weight). Normal liver content was found in patients with acute hepatitis, steatosis of the liver, hepatic amuloidosis, haemochromatosis, and Gilbert's syndrome. The urinary copper excretion was increased (is greater than 75 mjg/24 h) in half the patients with primary biliary cirrhosis and occasionally in the other patient groups. Serum ceruloplasmin was raised in more than half of all patients, and none had levels below the reference range. Raised heaptic copper content did not always coincide with enhanced urinary copper excretion, but was significantly correlated with this parameter and also with ceruloplasmin, alkaline phosphatases, and vitamin-K-dependent clotting factors, but not with ALAT. Combination of laboratory data, as found in typical cases of hepatolenticular degeneration, was not observed in this study, including 66 patients.
 ...
PMID:Hepatic copper content, urinary copper excretion, and serum ceruloplasmin in liver disease. 83 74

Kayser-Fleischer rings are pigmented corneal rings at the limbus of the cornea in Descemet's membrane that have been deemed pathognomonic of Wilson's disease. However, we have observed four exceptions in patients with non-Wilsonian liver disease. Three patients had primary biliary cirrhosis and one patient had chronic aggressive hepatitis with cirrhosis. Pigmented corneal rings were seen only by slit-lamp examination. Hepatic, serum, and urinary copper and serum ceruloplasmin levels were significantly elevated in the patients with primary biliary cirrhosis. Radiocopper (64Cu or 67Cu) studies in patients with primary biliary cirrhosis showed plasma disappearance curves which allowed a clear distinction from Wilson's disease in that all three patients with primary biliary cirrhosis showed a secondary rise in radiocopper that presumably represented copper incorporation into ceruloplasmin. In one patient, in whom 64Cu in ceruloplasmin was studied specifically, incorporation was found to be normal.
 ...
PMID:Pigmented corneal rings in non-Wilsonian liver disease. 84 86

After a short survey on the history of the therapy of liver cirrhosis the importance of the prevention of alcoholism is emphasized. A "liver diet" is regarded as unnecessary, prednisolone is recommended for the treatment of the active cirrhosis. After a short description of the principles of the therapy of biliary cirrhoses, of haemochromatosis and of Wilson's hepatocerebral degeneration the use of restriction of salt, saluretics and aldosterone antagonists in the treatment of ascites is discussed in detail. After description of the conservative therapy of the haemorrhage from varices with the compression sound, intraarterial octapressin infusion and combat against consumption coagulopathy finally the prophylaxis of the hepatoportal encephalopathy with reduction of the protein intake and the restriction of the formation of toxic products of protein metabolism in the intestine by application of neomycin or lactulose, respectively, is described.
 ...
PMID:[Conservative therapy of liver cirrhosis]. 85 44

In two sibships 7 of 24 siblings were homozygous for Wilson's disease. In family A, the largest kindred of this recessively inherited disease thus far reported, the proband presented with chronic active hepatitis, one sibling died of cirrhosis, a second had clinical evidence of chronic liver disease and two others had biochemical and histologic changes in liver biopsy specimens. In family B the proband had cirrhosis and portal hypertension and one sibling had biochemical and histologic evidence of liver disease. All six living patients had low serum concentrations of ceruloplasmin and copper and a high 24-hour urinary excretion of copper, which was greatly increased by administration of D-penicillamine. None showed neurologic abnormalities and only one had Kayser-Fleischer rings (detectable only by slit-lamp examination). Each patient had an erythrocyte sedimentation rate (ESR) of 8 mm/h or less. After 3 and 2 years, respectively, of D-penicillamine therapy the conditions of the two probands had improved. Liver function became normal in three siblings, and no abnormalities developed in the remaining one. Thus, since Wilson's disease may present with chronic active hepatitis or cirrhosis with a normal ESR and without ocular or neurologic signs, it may be a more common cause of liver disease in young people than has been appreciated.
 ...
PMID:Wilson's disease: a common liver disorder? 86 10

A 32-year-old woman was found to have chronic active hepatitis and cirrhosis after exploratory celiotomy resulted in hepatic decompensation. Subsequent investigation confirmed the diagnosis of Wilson's disease. This case demonstrates that Wilson's disease may manifest itself as chronic active hepatitis as late as the fourth decade of life without neurologic symptoms or findings. Wilson's disease should be actively considered in patients with chronic active hepatitis or cirrhosis, even in older age groups and despite the absence of central nervous system manifestations.
 ...
PMID:Chronic active hepatitis and cirrhosis in Wilson's disease. 87 27

In 19 patients with Wilson's disease we found an increased humoral immune response, i.e. a higher level of IgG and IgM, a higher titre of antibodies against Kunin's CA antigen and a depressed cell-mediated immunity i.e. a lower response to DNCB and E. coli in skin tests, lower lymphocyte transformation when stimulated by Con A, PPD, Candida albicans and streptokinase and a lower production of macrophage migration inhibition factor. The changes observed in the group of patients with liver cirrhosis caused by other facotrs than Wilson's disease were similar but less pronounced. We also found that leukocytes of patients with Wilson's disease have an impaired bactericidal activity and that copper ions have an inhibitory effect on some tests for cell-mediated immunity. It seems probable that immunological abnormalities in Wilson's disease are caused by liver cirrhosis but we cannot exclude an inhibitory effect of copper ions upon the immune response and an associated effect upon leukocyte metabolism.
 ...
PMID:Immunological observations on patients with Wilson's disease. 97 18


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