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)

Immunocytochemical staining patterns for glial fibrillary acidic protein (GFAP) and S-100 protein (S100P) were compared in cerebral cortex, basal ganglia and white matter of eight cases with hepatic encephalopathy (HE), including four cases of Wilson's disease and four of liver cirrhosis, and of eight age-matched controls, using the peroxidase-antiperoxidase method on adjacent paraffin sections. The majority of Alzheimer type II glia (Alzg II) showed prominent immunoreactivity for S100P but not for GFAP, resembling normal astrocytes of protoplasmic type; Alzg II might be interpreted as being peculiar types of reactive astrocytes retaining characteristics of protoplasmic astrocytes. A small number of Alzg II cells showed slight perinuclear immunoreactivity for GFAP; some lacked both markers. This suggests a spectrum of metabolic changes in these two proteins in Alzg II. GFAP-positive Alzg II cells were restricted to basal ganglia and white matter adjacent to grey matter, indicating that expression of GFAP in Alzg II might be modulated by local factors. Alzheimer type I cells and Opalski cells in Wilson's disease were immunoreactive for both proteins, confirming their astroglial origin and different character from that of Alzg II. In morphometric comparison, the proportion of GFAP-positive glial cells decreased in the cortex (P less than 0.001) but not significantly in the white matter (0.05 less than P less than 0.1), confirming earlier data that the prominent reduction of GFAP in HE brains is restricted to the grey matter.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Glial fibrillary acidic protein and S-100 protein in human hepatic encephalopathy: immunocytochemical demonstration of dissociation of two glia-associated proteins. 372 31

Evaluation of the literature concerning the various approaches for the treatment of Wilson's disease led to the conclusion that zinc sulphate might be a good choice because it is effective and relatively safe. Twenty seven patients were managed with zinc sulphate for a total period of 142 patients-years. The drug was administered in doses varying from 300 to 1200 mg/day. Of the 9 patients who were treated with zinc from the start, 8 improved and one died from severe cirrhosis. All 8 patients who were placed on zinc after intolerance to penicillamine did well on zinc therapy. Ten patients were changed to zinc after they had first been treated with penicillamine without developing signs of intolerance. Of this group 8 patients were kept on long-term zinc therapy, 2 were changed back to penicillamine because of personal preference. Signs of intolerance to zinc were not observed. All patients kept a diet containing about 1.2 mg of copper a day. Our experience supports the idea that zinc sulphate is a good choice for the treatment of Wilson's disease: the drug is effective, safe and cheap.
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PMID:Management of Wilson's disease with zinc sulphate. Experience in a series of 27 patients. 381 64

The weights of the spleens of series of patients with various disorders of children dating from birth or early infancy and causing splenomegaly, with or without cirrhosis of the liver, were analyzed. The linear regression equation for spleen weight versus age in months for each disease was derived, and the rate constants from these equations were adjusted for the age range of the patients in each group. The original data of Coppoletta and Wolbach were used for normal values. The rates of splenic growth of appropriate entities for which the regression equation could be computed fell into three groups, with adjusted rate constants (growth of spleen in grams per month) of 6.53-6.95 (biliary atresia, thalassemia, and cirrhosis following neonatal hepatitis), 2.30-2.62 (cirrhosis of alpha-1-antitrypsin deficiency, infantile polycystic disease, and spherocytosis), and 1.06-1.11 (cystic fibrosis and idiopathic thrombocytopenic purpura). These classes of splenic growth rates are approximately 10, 3.7, and 1.6 times the normal growth rate (0.67 g/mo). Rate constants could not be computed for the categories cirrhosis following viral hepatitis and hemolytic anemia other than spherocytosis and sickle cell anemia, and the numbers of patients with splenic vein obstruction, cirrhosis with the cholestatic syndrome of parenteral alimentation, hypoplastic anemia with hemosiderosis, tyrosinemia, Byler's disease, congenital hepatic fibrosis, and Wilson's disease were too few for analysis. The significance of the finding of classes or "quantum groups" of splenic growth rates in disorders of children, dating from birth or early infancy and causing splenomegaly, is uncertain. Comparable data on adequate series of patients with other appropriate disorders will be necessary.
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PMID:Splenic growth rates in cirrhotic and other splenomegalic diseases of childhood. 384 62

The lack of information on survival in young adults with cirrhosis and the increasing use of liver transplantation in this age group have led us to carry out a retrospective analysis of the clinical course and survival in 83 young adults aged between 15 and 30 years presenting to the Liver Unit between 1970 and 1983. Fifty four (65%) patients had cirrhosis at initial presentation and in the remaining 29 (35%) this developed within the study period. The overall five year survival of the group, excluding 14 cases treated by transplantation, was 70%. When considered according to aetiological groups this was 83% in those with chronic active hepatitis, 60% in those with cryptogenic cirrhosis and 37% in Wilson's disease. When considered in relation to Child's grading, only three deaths occurred in the 45 patients with well-compensated liver disease (Child's grade A and B). Of the 38 patients with Child's grade C, 20 (83%) of the 24 patients not undergoing transplantation have died, whereas eight (57%) of the 14 receiving liver grafts are alive and well.
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PMID:Clinical course of cirrhosis in young adults and therapeutic potential of liver transplantation. 391 May 22

Primary sclerosing cholangitis is a chronic, cholestatic syndrome characterized by fibrosing inflammation of the bile ducts that may lead to cirrhosis and death from liver failure. Previous reports have suggested abnormal hepatic copper metabolism in this disease. Therefore, in 70 patients, we prospectively determined the levels of hepatic copper, serum copper, and serum ceruloplasmin, and the rate of urinary copper excretion to assess the diagnostic and prognostic usefulness of these tests. Virtually all patients had at least one abnormal copper test. Hepatic copper levels were elevated in 87% of patients [292 +/- 38 micrograms/g dry wt (mean +/- SE)] and 24-h urinary copper levels in 64% of patients [135 +/- 15 micrograms/24 h (mean +/- SE)] to values comparable to those seen in Wilson's disease or primary biliary cirrhosis. In advanced histologic stages of primary sclerosing cholangitis, progressively higher mean levels of hepatic and urinary copper were found. In the liver, mean copper content (in micrograms per gram dry weight) in disease stages I and II was 147 +/- 36 (mean +/- SE); in stage III (fibrosis), 302 +/- 68; and in stage IV (cirrhosis), 379 +/- 69. In the urine, mean copper excretion (in micrograms per 24 h) in stages I and II was 72 +/- 14 (mean +/- SE); in stage III, 100 +/- 14; and in stage IV, 207 +/- 30. Higher hepatic and urinary copper levels at initial evaluation were associated with decreased survival during a median follow-up period of 2.6 yr: patients with hepatic copper greater than 250 micrograms/g dry wt and urinary copper excretion greater than 200 micrograms/24 h at initial evaluation had an 18-mo survival of less than 60%. We conclude that abnormal copper metabolism is a universal feature of primary sclerosing cholangitis, that hepatic copper accumulates and urinary copper excretion increases as the disease progresses, and that the hepatic copper concentration and the 24-h urinary copper determination are useful prognostic indicators in this disease.
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PMID:Abnormalities in tests of copper metabolism in primary sclerosing cholangitis. 400 18

The patterns and incidences of orcein-positive granules of copper-binding protein (CBP) in 2,531 liver biopsy specimens from children and adults with a large variety of liver diseases are reported. Fetal and neonatal livers have high physiologic levels of copper and CBP, which fall to within the adult range by the third to the sixth month of life. Therefore, in liver specimens from children less than 6 months of age, it was not possible to determine whether the orcein-positive granules present represented physiologic or pathologic deposits of CBP. In adults and in children older than 6 months of age, CBP granules were found almost exclusively in association with four main groups of liver diseases: Wilson's disease, chronic biliary disorders, cirrhosis/extensive fibrosis, and primary liver tumors. Orcein-positive granules were never found in patients with acute liver disease. The granules were extremely helpful in distinguishing chronic biliary diseases from acute cholestatic and hepatic disorders, primary biliary cirrhosis from chronic active hepatitis, and primary liver tumors from metastatic tumor deposits.
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PMID:Copper-binding protein in liver cells. 400 43

Recently Brewer et al. reported the possibility of an oral zinc therapy in Wilson's Disease. We treated a 19 years old patient with decompensated liver cirrhosis due to Wilson's disease with zinc-sulphate. D-Penicillamine had to be withdrawn since proteinuria occurred under treatment. After the discontinuation of D-Penicillamine an increase of serum copper almost up to normal range was observed; concomitantly urinary copper elimination decreased. Under oral zinc sulphate therapy (145 mg/day) a drop of serum copper level was achieved and liver function improved: serum albumin, gamma globulins and prothrombin time reached normal values. The patient did not complain any side effects during oral zinc sulphate therapy. Oral zinc therapy in Wilson's Disease may be regarded as an alternative to D-Penicillamine treatment when this drug has to be discontinued because of side effects.
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PMID:[Oral zinc in Wilson disease--an alternative to D-penicillamine]. 406 Jul 99

Thirteen patients with Wilson's disease were compared with seven cirrhotic and 13 normal controls to define better the haematological abnormalities in this condition. Hypersplenism (anaemia, leukopenia, thrombocytopenia, and reduced red cell survival) commonly occurred in patients with both Wilson's disease and cirrhosis. These abnormalities correlated with splenic enlargement. Despite reduced haematocrits, red cell mass was greater in these two groups than in normal controls. Plasma volume and the body haematocrit/peripheral haematocrit ratios were also greater in patients with Wilson's disease and cirrhosis. Increased splenic sequestration of (51)Cr-tagged red blood cells was not demonstrated in any subjects. The hypersplenism in patients with Wilson's disease is similar to that found in patients with cirrhosis from other causes.
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PMID:Hypersplenism in Wilson's disease. 502 27

A case of acquired hepatocerebral degeneration secondary to biliary cirrhosis is described. It differs from the conventional type because of the clinical predominance of cerebellar symptomatology and because the principal neuropathological abnormalities were restricted to the pallidum and to the cerebellum.
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PMID:Acquired hepatocerebral degeneration: report of an atypical case. 547 47

The clinical course of certain patients with Wilson's disease resembles that of patients with viral or drug-induced fulminant hepatitis lasting only few weeks from recognition of symptoms to severe hepatic insufficiency and death. The disease is complicated by hemolysis and is characterized by hypercupremia. Routine laboratory findings may underestimate the severity of the disease. These patients, as well as patients with decompensated Wilsonian cirrhosis who are not responding to therapy, should be considered as candidates for liver transplants.
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PMID:Wilson's disease: indications for liver transplants. 636 55


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