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)

The concentration of adenosine 3':5'-cyclic monophosphate in the spinal fluid of ten patients with liver dysfunction was analyzed. Ages of the patients ranged from 31 to 75 years. The state of consciousness varied between normality and stupor. After a liver biopsy the diagnoses were as follows: cirrhosis in six cases, porphyria cutanea tarda in one case, hepatic metastases in two cases and Wilson's disease in one case. Mean values in these patients (22.91 +/- 4.18 pM/ml) have been significantly greater (p less than 0.0005) than those in ten control individuals (15.55n control individuals (15.44 +/- 3.66 pM/ml). Values corresponding to two patients in coma were still higher (52.62 and 36.50 pM/ml respectively). A previous lumbar puncture carried out in one of these patients when he was conscious showed a figure of 23 pM/ml. These results suggest a progressive rise of cyclic adenosine monophosphate in the spinal fluid in relation to clinical impairment, and may indicate a similar behaviour for this nucleotide to that of tryptophan, as reported by other authors. These findings point toward the role of the alteration of neurotransmitters in the pathogenesis of hepatic coma.
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PMID:[Cyclic adenosine monophosphate in the cerebrospinal fluid of patients with liver disease (author's transl)]. 737 36

During 1965 to 1968, 80 workers who had been engaged in the production of 2, 4, 5-sodium trichlorphenoxyacetate and butylester of trichlorphenoxyacetate acid became ill. The cause of the illness was 2, 4, 7, 8-tetrachlordibenzo-p-dioxin. A 10-yr study has been conducted for 55 exposed individuals. The majority of the patients developed chloracne, and 11 manifested porphyria cutanea tarda. Approximately one-half of the patients suffered from metabolic disturbances, i.e., pathologically elevated lipids with abnormalities in the lipoprotein spectrum, and two-fifths of the patients had pathological changes in the glucose tolerance test. One-third of the patients had biochemical deviations indicative of a mild liver lesion. Histological examination revealed light steatosis, or periportal fibrosis, or activation of Kupffer cells. Fluorescence of the liver tissues was present in ultraviolet light. In 17 persons symptoms of nervous system focal damage existed, with predominance of peripheral neuron lesion of the lower extremities (verified by EMG examination). The majority of patients suffered from various psychological disorders. As of this date, two patients have died of bronchogenic lung carcinoma; one of liver cirrhosis; one of a rapidly developed, extremely unusual type of atherosclerosis precipue cerebri; and two patients have died in traffic accidents. The conditions of most other patients have improved.
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PMID:The development and prognosis of chronic intoxication by tetrachlordibenzo-p-dioxin in men. 746 93

Chronic hepatic porphyrias (CHP) are associated with different degrees of liver damage. They range from minimal histological changes to cirrhosis and primary liver carcinomas. Forty patients with early stages of porphyrias were compared to 85 patients with clinically manifest porphyrias, Porphyria cutanea tarda. This comparison resulted in the fact that cirrhosis occurred more often in early (or latent) stages (65%) than in PCT (31%) confirmed by laparoscopy. Thus the severity of the liver disease does not depend on the duration and intensity of cutaneous symptoms or pathobiochemical syndromes but they more often depend on the long-term influence of so-called trigger factors, among them above all alcohol and iron overloading. We conclude that the proof of latent CHP has an index function for severer liver damage.
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PMID:[Liver cirrhosis and chronic hepatic porphyria]. 748 90

The authors consider two groups of patients with overt sporadic porphyria cutanea tarda (PCT) from different continents, with the aim of evaluating the possible impairment of the liposoluble antioxidative system, given the possible synergic effect of porphyrins and iron in promoting oxidative cellular damage. Twenty-three Italian outpatients with overt sporadic PCT and 11 outpatients with PCT from Buenos Aires (Argentina) were matched with 60 patients with liver cirrhosis and 52 healthy Italian controls. Serum levels of alpha- and beta-carotene, cryptoxanthin, zeaxanthin, lutein, lycopene, retinol and alpha-tocopherol were detected by a high-performance liquid chromatographic technique devised in our laboratory, which afforded an accurate and simultaneous resolution of all these compounds. The results point to a significant reduction in plasma levels of alpha- and beta-carotene in both the PCT populations with respect not only to controls, but also to the cirrhotic population, which had more severe liver damage. Moreover, other carotenoids with proven antioxidative properties, like cryptoxanthin and lycopene, are greatly reduced in our PCT populations. This confirms the suggested synergic effect of iron and porphyrins in the oxidative intracellular damage with consequent depletion of antioxidative liposoluble molecules.
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PMID:Liposoluble vitamins and naturally occurring carotenoids in porphyria cutanea tarda. 755 69

A common characteristic of patients with porphyria cutanea tarda (PCT) is liver dysfunction. The degree of liver damage is usually mild but liver cirrhosis is a common associated finding. Hepatic iron overload is a characteristic feature but the role of iron in the pathogenesis of the condition has not yet been clarified. Heavy alcohol consumption is a frequent precipitant of PCT. A strong association with markers of past hepatitis B virus infection was first demonstrated but a high prevalence of hepatitis C virus markers is now noted. Although the potential to give rise to hepatocellular carcinoma (HCC) in PCT patients with cirrhosis is well recognized, HCC itself has a risk of developing PCT. HCV or the cirrhotic change itself has been attributed as the risk factor for the occurrence of HCC.
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PMID:[Association of cirrhosis and hepatocellular carcinoma with porphyria cutanea tarda]. 761 64

The causes of liver disease, ranging from fatty changes to cirrhosis and hepatocellular carcinoma, in porphyria cutanea tarda (PCT) remain unclear. We tested 100 consecutive PCT patients for antibodies to hepatitis C virus (HCV) by enzyme-linked immunosorbent assay and a recombinant immunoblot assay. 75 (79%) patients with sporadic PCT but none of 5 with familial PCT were positive. HCV RNA was found in serum of all 18 anti-HCV-positive patients tested. There were no significant differences in the prevalence of anti-HCV between treated and untreated patients or between those with and without various HCV risk factors. The frequency of anti-HCV increased with the severity of liver histology. These findings implicate HCV in the aetiology of PCT-associated liver disease.
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PMID:Is hepatitis C virus infection a trigger of porphyria cutanea tarda? 768 54

The recent identification of the hepatitis C virus and development of assays to detect antibodies to hepatitis C virus has allowed assessment of the prevalence of hepatitis C virus infection in patients with a variety of liver and other diseases. The aim of this study was to investigate the prevalence of hepatitis C virus antibodies and severity of liver injury in patients with porphyria cutanea tarda. Sixty-two patients were studied. Serum samples were analyzed for liver function parameters and markers of hepatitis B virus infection. Frozen serum samples from 34 patients with porphyria cutanea tarda, obtained when patients were seen at the hospital for the first time, were analyzed for hepatitis C virus antibodies with enzyme-linked immunosorbent assays (first- and second-generation) and a recombinant immunoblot assay. As controls, serum samples from 19,788 blood donors, 40 patients with alcoholic liver disease and 138 hospitalized patients without liver disease were also tested for hepatitis C virus antibodies. Liver biopsy was performed in 42 porphyria cutanea tarda patients. Specimens were evaluated for steatosis, siderosis, fibrosis, severity of inflammation and the presence of cirrhosis. In addition, the degree of necroinflammatory change and fibrosis were quantitated with the histologic activity index described by Knodell et al. The prevalence of hepatitis C virus antibodies in patients with porphyria cutanea tarda (62%) was higher than that in blood donors (0.79%), patients with alcoholic liver disease (17.5%) or hospitalized patients without liver disease (5.8%).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Hepatitis C virus antibodies and liver disease in patients with porphyria cutanea tarda. 753 99

Hepatocellular carcinoma probably represents the final step of the effects of the various precipitating agents for porphyria cutanea tarda, such as alcohol, drugs, hormones, and hepatitis C infection. Risk factors associated with its development include male gender, age over 50 years, liver fibrosis or cirrhosis, and a long history (over 10 years) of symptomatic porphyria cutanea tarda.
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PMID:The porphyrias and hepatocellular carcinoma. 771 39

Urinary and fecal levels of porphyrins were measured spectrophotometrically for 388 patients. 66 of them suffered from melanodermic skin lesions without hepatic affection, 95 had chronic hepatic diseases and 227 exhibited porphyria cutanea tarda. The results were considered in relation to the lesion and alcohol habits. Alcohol proved to provoke manifestations of porphyrin disbolism. High protoporphyrin fecal concentrations serve early indications of alcohol-induced damage to the liver. Alcohol abuse results in persistent disorders of porphyrin metabolism in subjects with chronic active hepatitis and hepatic cirrhosis. In established clinical and biochemical syndrome of porphyria cutanea tarda alcohol contributes to further progression of fermentopathy specific for relevant porphyria.
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PMID:[Alcohol and its effect on porphyrin metabolism]. 790 74

The need for accurate and noninvasive evaluation of liver iron stores prompted us to evaluate the reliability of high-field magnetic resonance imaging equipment in liver patients with low or moderate siderosis, given the poor results obtained using systems operating at low field strength in such cases. Twenty patients with sporadic porphyria cutanea tarda and 28 with comparable chronic liver diseases (chronic hepatitis or cirrhosis) and moderate siderosis were compared with 10 patients with idiopathic or secondary hemochromatosis and 10 healthy controls. Plasma iron profile, ferritin concentration and liver iron concentration, determined with atomic absorption spectroscopy, were matched with the magnetic resonance parameters-namely, transverse relaxation time and the signal intensity for a given proton amount, obtained with equipment operating at a field strength of 1.5 T. Hemochromatosis patients with mean liver iron concentrations of 550 mumol/gm dry wt (vs. 10 mumol of controls) exhibited an impressive reduction in the signal intensity with respect to the other three groups, and this reduction prevented any further comparison with the same porphyria cutanea tarda and chronic liver disease groups, whose liver iron level was twice that of the controls. The signal intensity remained almost unchanged in the latter groups, whereas the transverse relaxation time was significantly reduced. Moreover, correlation with liver iron was significantly inverse in the case of the transverse relaxation time (n = 17, r = 0.62, p = 0.008) and direct in the case of the transverse relaxation rate. The transverse relaxation time values returned to normal in five patients who had completed an iron-depletion program.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Magnetic resonance imaging and different levels of iron overload in chronic liver disease. 851 72


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