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

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

From over 800 patients with chronic hepatic diseases admitted to the Institute of Internal Medicine between 1960-1969 and in whom diagnosis was assessed by hepatic biopuncture, 180 were reexamined 3-14 years later to estimate the clinical evolution and the prognostic value of the bioptic findings. The evolution was favourable in most of the cases of persistent chronic hepatitis (70%) and of moderate aggressive chronic hepatitis (59%). Severe aggressive chronic hepatitis evaluated favourably in 34% of the cases and was aggravated in 42% of the cases (26% exits). In the patients with cirrhosis the percentage of deaths reached 65 but 30% were still in a stationary form; a single exceptional case was improved 7 years after the hepatic biopuncture. The prognostic value of hepatic biopuncture is much increased if, in addition to the common histological features, other histochemical and morphological criteria such as: structural stability of collagen fibers, importance of cellular infiltrates and presence of plasma cells, nuclear changes, degree of steatosis, amount of glycogen storage, siderosis and cholestasis are also taken into consideration.
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PMID:Primary evolution of chronic hepatitis estimated 3 to 14 years after the liver biopuncture. 124 40

In 28 out of 40 patients with idiopathic haemochromatosis manifest diabetes mellitus could be demonstrated 19 patients required insulin. Treatment of diabetes with or without insulin was problem-free. In only two patients there was an insulin resistance which required high doses of insulin some of the time. There was a family history of diabetes in eleven patients. Minimal diabetic retinopathy in two patients was the only typical complication specific to diabetes. Severe forms of microangiopathy are seldom seen in haemochromatosis diabetes. This form of diabetes is probably mainly of genetic origin. Liver cirrhosis and fibrosis and possibly pancreatic siderosis are additional factors to be considered. A sufficiently long and intensive venesection treatment leads to clinical improvement in the diabetes in only a small fraction of the haemochromatosis patients.
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PMID:[Diabetes mellitus in idiopathic haemochromatosis (author's transl)]. 127 43

Thirty-eight patients with porphyria cutanea tarda (PCT) have been seen in the last 18 years. Five of these patients (13%) developed hepatocellular carcinoma (HCC) during follow-up. We analyzed the differences in clinical, laboratory and liver histology findings at presentation, between patients who developed HCC during follow-up (HCC-group, n = 5) and those who did not (PCT-group, n = 33). Of the clinical features the duration of skin-symptoms was longer in the HCC-group (mean: 10.4 +/- 1.1 years) than in the PCT-group (mean: 1.4 +/- 1 years) (p less than 0.001). No differences in routine laboratory findings were found. Although 11/38 (29%) patients had serologic evidence of a past hepatitis B virus infection and 7/38 (18%) patients had antibodies against hepatitis C virus, no differences in these parameters were found between the PCT-group and the HCC-group. In all 34 liver biopsies a variable degree of siderosis was found (PCT-group vs. HCC-group: NS). Only piecemeal necrosis (p less than 0.01) and advanced fibrosis or cirrhosis (p less than 0.001) were more common in liver biopsies in the HCC-group. In conclusion, factors related to an increased risk of HCC in PCT are: a) a long symptomatic period before start of treatment and b) the presence of chronic active hepatitis and/or advanced fibrosis or cirrhosis in liver biopsies.
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PMID:Hepatocellular carcinoma in porphyria cutanea tarda: frequency and factors related to its occurrence. 132 Jan 75

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

Congenital dyserythropoietic anaemia type II, or HEMPAS (hereditary erythroblastic multinuclearity with positive acidified serum lysis test) is a genetic disease caused by membrane disorganization of erythroid cells. The primary defect of this disease lies in the gene encoding enzyme(s) which is responsible for the biosynthesis of Asn-linked oligosaccharides chains of glycoproteins (Fukuda et al, 1990). In order to know whether this gene defect affects the glycosylation in the cells other than the erythroid cells, the carbohydrate structures of the transferrin isolated from the sera of HEMPAS patients were analysed. Fast atom bombardment mass spectrometry analysis showed the presence of high mannose type and hybrid type oligosaccharides in the HEMPAS transferrin which is in contrast to the complex-type oligosaccharides found in the normal transferrin. The results strongly suggest that biosynthesis of Asn-linked oligosaccharide chains in HEMPAS hepatocytes is disturbed. As a result, the serum glycoproteins with incompletely processed carbohydrates are circulating in the plasma in HEMPAS patients, but they must have been absorbed by the cells in the liver and the reticuloendothelial cells. Upon intravenous infusion into rats, as much as 30% of the HEMPAS transferrin was cleared from the plasma circulation. The majority of the HEMPAS transferrins was taken up by the liver, and transferrin was distributed both in the hepatocytes and the Kupffer cells. The presence of enormous amounts of aberrantly glycosylated serum glycoproteins may lead to the liver cirrhosis and secondary tissue siderosis seen in HEMPAS patients.
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PMID:Incompletely processed N-glycans of serum glycoproteins in congenital dyserythropoietic anaemia type II (HEMPAS). 148 62

Iron-deficient female Wistar rats were fed a diet, which contained 0.5% trimethylhexanoylferrocene, over a 56-week period. This dietary iron loading resulted in a progressive siderosis and enlargement of the liver with a maximum iron content of 947.0 +/- 148.0 mg (vs. 0.07 +/- 0.04 mg in iron deficiency) and a maximum organ weight of 39.4 +/- 6.6 g (vs. 6.9 +/- 1.4 g in iron-deficient control rats). Up to 43 weeks, whole liver iron rose by increase in iron concentration (max. 28.0 +/- 6.1 mg/g wet weight, w.w.) as well as by enlargement of the organ. Afterwards whole liver iron increased solely by ongoing hepatomegaly. At the commencement of iron loading, stainable iron was almost exclusively stored by hepatocytes equally throughout all areas of the liver lobule. Later, the distribution of iron-loaded hepatocytes became strikingly periportal, and, in addition, Kupffer cells as well as sinus-lining endothelia began to store iron. Animals with a liver iron concentration of more than 10.4 +/- 0.75 mg/g w.w. showed no further increase in ferritin and haemosiderin within hepatocytes. Iron-burdened Kupffer cells/macrophages, however, accumulated permanently, hereby forming intrasinusoidal and portal siderotic nodules and areas. First signs of liver damage such as necrosis of single hepatocytes and mild fibrosis began at a liver iron concentration of 14.7 +/- 1.4 mg/g w.w. With advancement of iron loading, focal necrosis of hepatocytes and iron-burdened macrophages took place, and significant perisinusoidal as well as portal fibrosis developed. Cirrhosis, however, the final stage of impairment in iron overload of the liver in humans, could not be induced in this animal model up to now.
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PMID:Iron overload of the liver by trimethylhexanoylferrocene in rats. 159 22

Magnetic resonance demonstrated siderotic regenerating nodules in a patient with hepatocellular carcinoma with accompanying liver cirrhosis. The siderosis disappeared when iron deficiency anemia developed in association with biliary hemorrhage. Magnetic resonance was sensitive enough to follow changes in the siderosis. The mechanism of iron deposition in regenerating nodules that accompanied liver cirrhosis did not seem to be related to the severity of the liver damage.
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PMID:Siderotic nodules in hepatic cirrhosis disappearing after biliary hemorrhage: MR imaging. 165 91

In a 59-year-old patient presenting in October 1981 with pancytopenia, hairy cell leukemia was diagnosed. Splenectomy, followed by treatments with oncovin, lithium, and prednisone were essentially without effect. Up to July 1984 the patient had been regularly transfused with a total of 62 unit. In June 1984 he acquired a transfusion associated hepatitis C which followed a chronic course and resulted in biopsy proven cirrhosis in 1989. The patient became independent of transfusions in July 1984. Repeated blood counts have shown a complete hematologic remission which has now lasted nearly 6 years, whereas focal leukemic infiltrates have persisted in the bone marrow. The patient has tolerated 20 courses of erythropheresis performed because of biopsy proven severe hepatic siderosis without a fall in hemoglobin. It is suggested that the spontaneous long-lasting hematologic remission of hairy cell leukemia is due to endogenous interferon produced in the course of chronic hepatitis C. Low serum levels of interferon-alpha and -gamma were detected.
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PMID:[Peripheral spontaneous remission of hairy cell leukemia following transfusion-associated hepatitis C]. 190 85

Forty-two male patients with alcoholic liver disease were studied for iron status by indirect hematological assays, including red cell ferritin (RCF), and histochemical estimations. Serum iron and ferritin, total iron-binding capacity levels were unrelated to iron deposits, whereas RCF concentration was a good index of iron stores as detected by direct assessment on bone marrow and liver biopsy specimens. A relatively high proportion of alcoholics (19%) were iron-deficient. Alcoholic patients with cirrhosis exhibited higher RCF values than patients with alcoholic hepatitis. However, this increase was apparently unrelated to cirrhosis per se. In alcoholics we found that RCF was mainly related to levels of bone marrow iron. The increased RCF values observed in patients with hepatic siderosis was mediated by marrow iron stores. RCF can therefore be regarded as a useful test to distinguish patients with liver siderosis and normal values of bone marrow iron.
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PMID:Evaluation of iron stores in patients with alcoholic liver disease: role of red cell ferritin. 245 66


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