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

Rare cases of hemochromatosis have been reported in patients who underwent prolonged oral iron therapy for hemolytic anemia or prolonged self-treatment with iron pills. A proportionately large segment of the South African Bantu tribe, who ingest large quantities of an alcoholic beverage brewed in iron pots, are found to have the disease. Reports of health fadists developing hemochromatosis due to excessive dietary iron intake, however, are extremely rare. This report presents clinical considerations and pathologic findings in a compulsive health fadist who consumed large numbers of vitamins containing iron. Clinical findings included the development and progression of cirrhosis of the liver, bronzing of the skin, and diabetes mellitus, all consistent with a diagnosis of hemochromatosis. Light microscopy of liver biopsies taken late in the course of the disease revealed a massive buildup of iron in the hepatocytes, less in the Kupffer cells, and sparse deposition in the epithelial cells of the bile duct. Minimal periportal fibrosis was noted. Electron microscopy showed numerous pleomorphic siderosomes with varying degrees of crystallization and ferritin attached at uniform intervals to the membranes of residual bodies. Abundant free ferritin was observed in most cells. The aggregated and membrane-associated ferritin was verified by non-dispersive x-ray analysis. An additional finding, noted only by electron microscopy, was the presence of many fat-storing cells of Ito, which are thought to be involved in the onset of fibrosis.
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PMID:Hemochromatosis caused by excessive vitamin iron intake. 47 11

A Hungarian family with four heterozygotes for Hb Lepore Washington is described. One, a 43-year-old male, had high levels of serum iron, saturated iron-binding capacity, and ferritin, and normal levels of folic acid and vitamin B12. Liver biopsy showed slight cirrhosis and marked iron deposition in parenchymal cells and in cells of the reticuloendothelial system. Heavy iron deposition was also found in the bone marrow. The patient is not an alcoholic and has no disease that requires blood transfusion. The hemochromatosis thus seems to be of idiopathic nature.
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PMID:Hemoglobin Lepore Washington and hemochromatosis in a Hungarian patient. 47 79

Deferoxamine-chelatable iron was measured in 103 patients with known or suspected iron overload. All of 34 patients with untreated hemochromatosis had distinctly elevated values for deferoxamine-chelatable iron. The mean value in these cases was significantly greater than that in patients with cirrhosis, who had little or no stainable hepatic iron. In 15 patients with hemochromatosis who were tested sequentially during the course of phlebotomy therapy, deferoxamine-chelatable iron proved a reliable index of the degree of reduction of storage iron. In 22 additional patients with partially treated hemochromatosis and 14 with iron overload accompanying chronic anemia, this test correlated well with the magnitude of iron deposits in liver or bone marrow. In patients with unexplained elevations of serum iron, normal or only slightly elevated deferoxamine-chelatable iron correctly indicated that storage (hepatic) iron was not excessive. The test was more reliable than determination of serum iron or transferrin saturation as an indicator of increased storage iron. Elevated values could not be attributed to disturbed liver function. Determination of deferoxamine-chelatable iron is a safe, practical, and useful procedure for identifying persons with increased iron stores and for assessing the effect of phlebotomy therapy.
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PMID:Deferoxamine-chelatable iron in hemochromatosis and other disorders of iron overload. 62 26

The value of tests for the detection of body iron overload was investigated in 8 patients with clinically manifest primary hemochromatosis, 12 patients with cirrhosis and iron overload and 20 patients with liver disease and low or normal iron stores. Iron overload was defined as the presence of stainable iron in more than 50% of hepatocytes in a liver biopsy specimen. The percentages of patients with a true-positive (abnormal) or true-negative (normal) result were: serum iron concentration 65%, transferin saturation 85%, serum ferritin concentration 78%, serum ferritin:serum glutamic oxaloacetic transaminase (SGOT) index 78%, percent iron absorption 58%, percent iron absorption in relation to serum ferritin concentration 80% and percent iron absorption in relation to serum ferritin:SGOT index 93%. The calculated predictive value of a normal test result for the exclusion of iron overload in patients with liver disease, a group with an assumed prevalence of iron overload of 10%, was 98% to 99% for transferrin saturation and serum ferritin concentration used alone and 100% for these measures used together; the predictive value of an abnormal result for the diagnosis of iron overload was less than 50% for all of the above measures used alone or in combination. Hence, in patients with an increased serum ferritin concentration or transferrin saturation, or both, determination of the hepatocellular iron content of a specimen from a percutaneous liver biopsy is required for the diagnosis of iron overload.
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PMID:Diagnostic efficacy of tests for the detection of iron overload in chronic liver disease. 67 27

Concentrations of iron, copper and zinc were measured in postmortem specimens of liver, muscle and brain tissue by atomic absorption spectrophotometry. Concentrations varied markedly in normal liver specimens from person to person, but not in other organ specimens. Copper concentrations in specimens from cirrhotic livers were normal in most cases, however at the upper limit of normal in about 20% of the cases evaluated. Concentrations of zinc and iron were lower than normal in cirrhotic livers. Mineral concentrations were higher by a factor of 10-20 in liver specimens of patients with hemochromatosis or M. Wilson than in normals. Iron overloading as a consequence of therapy (secondary hemochromatosis) and liver changes due to longtime artificially elevated plasma copper levels may imitate the adult form of the "classic" mineral storage diseases mentioned. Thus "athrocytotic" liver cirrhosis, being caused or accompanied by metal excess has to be considered as a polyetiological syndrome.
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PMID:[Minerals in normal and cirrhotic liver. The pathology of mineral dependent athrocytic liver cirrhosis]. 73 12

Blood levels of estradiol, testosterone, luteinizing hormone (LH), follicle stimulating hormone (FSH), sex steroid binding globulin (SSBG), and free steroids were surveyed in 117 men to define the pattern of hormonal abnormalities and to examine the relationships between the hormone levels and the development of the endocrine features of cirrhosis. When compared with healthy men of similar ages, the patients had significantly lower metabolic clearance rates (p .001), testosterone production rates (p .001), total and free levels of testosterone (p .001), reduced testosterone responses to human chorionic gonadotropin (HCG) stimulation, higher estradiol, LH, and FSH levels, and higher binding capacities of SSBG. The metabolic clearance and plasma production rates of estradiol were not markedly different from those of controls. Severely ill patients with liver failure of hemochromatosis had low levels of LH and FSH respones to clomiphene and LH-releasing hormone. Patients with gynecomastia and spider naevi had higher estradiol levels than in those without these signs. Longitudinal studies indicated that the hormonal levels, endocrine features, and severity of the liver disease could change independently. It is concluded that the clearance of estradiol from plasma is normal in most patients with liver disease and that reduced degradation of estrogens is not the initial event in the sequence leading to the hormonal abnormalities of cirrhosis. Usual findings of liver failure are elevated gonadotropin levels and a poor Leydig cell response to HCG which suggest that the hypogonadism is primary in most patients with cirrhosis. Discrepancies in the expected relationships between the hormone and clinical changes suggest that other factors than those studied are also involved in the genesis of hepatic cirrhosis.
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PMID:A study of the endocrine manifestations of hepatic cirrhosis. 76 39

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.
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PMID:Hepatic copper content, urinary copper excretion, and serum ceruloplasmin in liver disease. 83 74

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.
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PMID:[Conservative therapy of liver cirrhosis]. 85 44

A lethal case of septicemia caused by Yershia enterocolitica serotype 3 is described. A 59-year-old male, previously healthy, presented with 6 weeks of fever, abdominal pains and gradual prostration ending in overhelming septicemia and death before a conclusive diagnosis was established. Necropsy showed cirrhosis, haemochromatosis and numerous abscesses in the liver.
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PMID:Fulminant septicemia caused by Yersinia enterocolitica. 90 87

A 68-year-old man, after having been diagnosed as having hepatic disease at about the age of 41 years, had been hospitalized frequently until his death. Blood sugar, iron, and copper had not increased during his illness. Although the diagnosis of liver cirrhosis had been made and he had been receiving therapy, various neurologic symptoms without disturbances of consciousness appeared six months before his death. Autopsy revealed hemochromatosis, liver cirrhosis, and pancreatic fibrosis. A large amount of iron had accumulated in the liver, the pancreas, and the thyroid gland, while considerable numbers of ceroid and lipofuscin pigment granules had accumulated diffusely in the brain. Abnormal astrocytes of the Alzheimer II type were diffusely distributed in the brain and contained no intranuclear glycogen which stained positive with the carmine stain. No spongy changes were seen in the deeper layers of the cerebral cortex. Chemical analyses for trace metals in the brain, liver, and kidneys revealed a large amount of iron and increased copper in the liver, and considerable quantities of copper, manganese, calcium, and mercury in the brain. Because of changes in the erythrocyte sedimentation rate and marked thymol turbidity seen before and after the occurrence of the neurologic symptoms, this man was suspected of having disorders of the trace-metal binding proteins and/or of their polymers.
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PMID:Hemochromatosis associated with brain lesions--a disorder of trace-metal binding proteins and/or polymers? 92 21


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