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

Because of uncertainty as to the molecular weight of transferrin, a previous comparison [Von der Heul et al., Clin. Chim. Acta 38, 347 (1972)] between transferrin content of serum and total iron-binding capacity cannot be definitive. We found a conversion factor for expressing the maximum amount of iron bound by 1 mg of transferrin. We compared the resulting calculated value with values obtained by three other methods for measuring total iron-binding capacity. We agree with the previous observation that the latter, as measured radioisotopically, give higher results than would be judged from the transferrin content but the same as those for two chemical methods. The diffusion rate of transferrin in agar was the same irrespective of the degree of iron saturation. Serum transferrin concentrations were low in patients with anemia resulting from malignancy, chronic disorders, and cirrhosis of the liver, and high or normal in patients with iron deficiency anemia and in pregnant women or women who were taking birth-control pills. Measurement of transferrin concentration can be used to distinguish iron deficiency anemia from anemia resulting from chronic disorders, but offers no advantages over existing methods for estimating total iron-binding capacity.
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PMID:Immunological measurement of transferrin compared with chemical measurement of total iron-binding capacity. 113 10

The relative distribution of storage iron between bone marrow and liver has not been adequately studied in patients with iron-loading disorders. To help clarify this we assessed iron metabolism in patients with iron overload and in control subjects with cirrhosis but no excess body iron. In 4 patients with advanced iron overload studied late in the course of their illness, excess hemosiderin was present in both bone marrow and liver, as expected. In contrast, 2 patients with idiopathic hemochromatosis whose excess iron had been depleted by phlebotomy subsequently developed progressive hepatic parenchymal and reticuloendothelial (RE) deposition of iron, yet marrow hemosiderin remained sparse. Moreover, surface radioactivity over the liver after an oral dose of 59Fe. These results suggest that during the initial stages of hemochromatosis there is a dissociation in the rate of iron accumulation between the bone marrow and liver. Excess hemosiderin appears to be deposited predominantly and preferentially in hepatic storage sites until the later stages of the disease.
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PMID:Distribution of storage iron as body stores expand in patients with hemochromatosis. 115 Nov 63

The interrelationships between liver iron concentration, the duration of iron-loading, and hepatic fibrosis, assessed morphometrically, have been studied in 32 specimens of liver obtained from 19 heavily transfused patients with thalassaemia major whose age ranged from 4 to 19 yr. Similar observations were made in a matched group of thalassaemic patients treated with long-term chelation therapy. The degree of liver damage ranged from very slight increase in fibrous tissue to severe fibrosis and cirrhosis. The severity of the fibrosis was closely correlated both with liver iron concentration and with age. While the relationship between fibrosis and age was linear, both the severity and the rate of fibrosis were exponentially related to liver iron concentration, damage accelerating as liver iron concentration exceeded 3 per cent, dry weight. By producing a modest but significant reduction in liver iron concentration chelation therapy resulted in a disproportionate but predictable retardation in the progression of the fibrosis. The factors affecting the distribution of iron between parenchymal and reticuloendothelial cells were also examined. In general stainable iron was uniformly distributed between parenchymal and reticuloendothelial cells from the early stages of iron-loading. Parenchymal siderosis was relatively heavier in splenectomised patients and in patients with liver iron concentrations above 3 per cent, dry weight than in non-splenectomised patients or patients with liver iron levels of less than 3 per cent, dry weight, but this did not affect the severity of the fibrosis. The relevance of these findings to the traditional concepts of the pathology of transfusional siderosis is discussed.
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PMID:Transfusional iron overload: the relationship between tissue iron concentration and hepatic fibrosis in thalassaemia. 115 28

Ferritins are iron-containing proteins found in normal tissues; they increase in concentration in many tumors and the blood of tumor-bearing individuals. We utilized a double-antibody radioimmunoassay for measurement of serum ferritin and defined the upper limit of normal as 146 ng/ml for women (mean 34 ng/ml) and 193 ng/ml for men (mean 93 ng/ml). Serum ferritin levels exceeded these limits in preoperative sera of 41% of women with mammary carcinoma (mean 199 ng/ml) and in 67% of women with locally recurrent or metastatic mammary carcinoma (mean 671 ng/ml). Individuals with hepatic inflammatory states are known to have high serum ferritin, and ferritin was increased in 43% of patients with hepatitis or cirrhosis (mean 364 ng/ml) and in 13% of patients with ulcerative colitis or gastroduodenal ulcers (mean 106 ng/ml). Measurement of serum ferritin may be useful in evaluation of patients with breast cancer and in monitoring their response to therapy.
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PMID:Measurement of serum ferritin by radioimmunoassay: results in normal individuals and patients with breast cancer. 118 3

While a number of studies investigated iron and copper storage or alpha 1-antitrypsin (A1AT) deficiency in the liver of patients with cirrhosis, we did not find any similar study in schistosomotic patients reported in literature. We investigated the storage of both metals and the A1AT deficiency in the liver of 72 cirrhotic and 27 schistosomotic patients (5 with the hepatointestinal and 22 with the hepatoesplenic form of the disease). Forty-four patients with cirrhosis were also alcoholic, and 28 were not. Iron storage was detected in 23 patients with cirrhosis (31.9%); among these 16 (36.3%) were alcoholic and 7 (25.0%) non-alcoholic (the difference was not statistically significant). Thirteen (56.5%), 5 (21.7%) and 5 (21.7%) patients presented I-grade, II-grade, and III-grade iron storage, respectively. Copperstorage was detected in 24 cirrhotic patients (33.3%), 15 being alcoholic (34.0%) in contrast with 9 non-alcoholic patients (32.1%), a statistically non-significant difference. A1AT deficiency was observed in 2 patients with cirrhosis (2.8%), one with history of alcoholism. HBsAg and HBcAg in hepatic tissue were detected in 5 cirrhotic patients (6.9%), three of them with a history of alcoholism. Iron and copper storage and A1AT deficiency were observed in 3 patients with cirrhosis (12.5%), while iron storage and A1AT deficiency were found in 2 additional patients with cirrhosis (2.8%). The authors underline that neither iron nor copper storage nor A1AT deficiency was found in any schistosomotic patient. The authors discuss the possible importance of these data.
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PMID:Iron, copper and alpha 1-antitrypsin deficiency in the liver of cirrhotic and schistosomotic patients. 130 14

Two hundred twelve Italian patients with genetic hemochromatosis (181 men, mean age 50 +/- 11 yr; and 31 women, mean age 49 +/- 10 yr) were followed for a median period of 44 mo (range = 3 to 218 mo). Alcohol abuse was present in 31 subjects (15%), and chronic HBV and HCV infection were seen in 19 (9%) and 35 (24%) of 145 cases tested, respectively. Twenty-four patients (11%) had concomitant beta-thalassemia trait. Liver biopsy revealed cirrhosis in 146 and a noncirrhotic pattern in the other 66. Perls' stain was degree III in 37 patients and IV in 171 patients. One hundred eighty-five patients underwent weekly venesection, and iron depletion was achieved in 122 cases after total iron removal of 3 to 41 gm. Death occurred in 44 patients after 3 to 198 mo and was due to hepatocellular carcinoma in 20 cases, liver failure in 10, extrahepatic cancer in six, heart failure in three and hemochromatosis unrelated causes in five. Cancer has developed in seven other patients still alive (hepatocellular in five and extrahepatic in two). No deaths were observed among noncirrhotic patients; cumulative survival rates in cirrhotic patients were 85%, 75%, 60% and 47% at 3, 5, 8 and 10 yr, respectively. Univariate analysis in the 146 cirrhotic patients showed that age greater than 60 yr, alcohol abuse, cardiomyopathy, skin pigmentation, portal hypertension, hypoalbuminemia, hypergammaglobulinemia and Child class B or C had significant negative prognostic value. At multivariate analysis, only alcohol abuse, gamma-globulins greater than 2.0 gm/dl and Child class B or C maintained their negative prognostic values (p less than 0.01, hazard ratio 2.7; p less than 0.001, hazard ratio 2.8; and p less than 0.001, hazard ratio 4.3, respectively).
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PMID:Survival and prognostic factors in 212 Italian patients with genetic hemochromatosis. 131 85

Fifty-six patients with idiopathic hemochromatosis (50 men and 6 women, mean age 49.7 +/- SD 11.1 and 60.8 +/- SD 10.3 years respectively) were studied and followed up for a median period of 44 months (range 3-168). At basal liver biopsy 44 patients had cirrhosis and 12 fibrosis. Iron depletion was achieved in 39 of the 46 cases who underwent iron removal by erythrocytapheresis. Eighteen patients died, 11 from malignancy and 7 from other causes. A total of 14 malignant neoplasms were observed (6 hepatocellular and 8 extrahepatic), of which 6 (5 hepatocellular and 1 pancreatic) were already evident at enrollment. Cumulative survival rates at 3, 5 and 8 years were 75.4%, 64.2% and 54.4% respectively, significantly lower than those in the general population. Probabilities of developing cancer in the 50 patients without cancer at diagnosis were 5.5%, 14.9% and 44.4% at 3, 5 and 8 years respectively.
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PMID:[Survival and development of neoplasms in 56 patients with idiopathic hemochromatosis]. 132 11

This is the second update of a study of 3,444 taconite miners and millers who were first exposed to taconite, with associated exposures to silica and nonasbestiform amphiboles, in the period 1947 through 1958. Previous analyses of deaths through 1977, and again through 1983, showed no significant excess deaths from any specific causes. The present study continues the follow-up through 1988, adding 14,748 person-years of observation and 261 death certificates for analysis. The population, reduced to 3,431 because of the detection of 13 earlier duplications, has now been observed for 101,055 person-years, with 1,058 deaths and 1,039 death certificates. Death certificates were obtained for 98.2% of those known to be dead. The total number of deaths was significantly fewer than expected. Based on US rates, the standardized mortality ratio (SMR) was 83 (ie, 83% of expected). Based on Minnesota death rates, it was 91. With both US and Minnesota death rates, the SMRs for malignant neoplasms, cancer of the respiratory tract, cancer of the digestive system, heart disease, nonmalignant respiratory disease, and cirrhosis of the liver were all below 100. Slightly elevated SMRs were found for cancer of the colon, cancer of the kidney, and lymphopoietic cancer. These elevations were not statistically significant. Separate analyses were made of total deaths, lung cancer deaths, and kidney cancer deaths in men who had worked with taconite for time periods of less than 1 year, 1-5 years, 5-10 years, and over 10 years, during observation periods less than 10 years, 10-20 years, and over 20 years.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:An updated study of taconite miners and millers exposed to silica and non-asbestiform amphiboles. 133 7

Mitochondrial cytopathies are multisystemic diseases of extremely variable expression caused by a deficiency in oxidative phosphorylation. Only five cases of neonatal liver failure in the context of mitochondrial cytopathy have been reported, with incomplete morphological data of the liver in three. In the case presented here, ascites had been diagnosed prenatally and liver failure was particularly severe (factor V less than 15% with fatal coma the fourth day). Histologically there were incomplete cirrhosis, microvesicular steatosis, major canalicular cholestasis with proliferative neocholangioles, and bile duct thrombi. There were also some iron pigments in the periportal area and partial glycogen depletion. By electron microscopy, mitochondria in numerous hepatocytes appeared abnormal with occasional cristae in a fluffy matrix, some containing dense inclusions. Study of respiratory chain activity showed a defect in cytochrome c oxidase (complex IV), revealed by oxygraphic measurement on fresh muscle biopsy and confirmed by spectrophotometric enzymatic assays performed on muscle and liver homogenates. The association of neonatal liver failure with hyperlactacidemia warrants investigation into a deficiency in oxidative phosphorylation.
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PMID:Fatal neonatal liver failure and mitochondrial cytopathy: an observation with antenatal ascites. 139 93

The relationship of pretreatment serum ferritin and hepatic iron concentration to body iron removed by venesections was evaluated in 33 patients with genetic hemochromatosis. The median values of the three variables considered were 1,950 micrograms/L (range = 255 to 10,000), 1,175 micrograms/100 mg dry weight (range = 270 to 4,310) and 10 gm (range = 2 to 41), respectively. At basal liver biopsy 18 patients had cirrhosis, 6 patients had fibrosis and 9 patients had a normal pattern; siderosis was degree 3 in 6 patients and degree 4 in 27 patients. The results of fitting a polynomial regression of second degree showed that the curve of serum ferritin on iron removed was a straight line (R2 = 0.79, with a significant coefficient of linearity, p less than 0.01, and a nonsignificant coefficient of curvature), whereas that of hepatic iron concentration on iron removed showed a curvature (R2 = 0.62, with significant coefficient of linearity and curvature, p less than 0.01) and reached a plateau. The sigmoid model fit the curve of hepatic iron concentration on iron removed (R2 = 0.61), which suggested a saturation of hepatic iron storage capability; the asymptote corresponded to a hepatic iron concentration of about 2,000 micrograms/100 mg. In alcoholic patients (17 cases) the location of the sigmoid was greater than in nonalcoholic patients. Our results suggest that iron deposition occurs in the liver before other organs are involved and that with massive iron overload hepatic deposits reach saturation, after which hepatic iron concentration does not always reflect the amount of total stores. Alcohol consumption could slow the saturation of hepatic iron deposits.
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PMID:Saturability of hepatic iron deposits in genetic hemochromatosis. 139 2


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