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)

Thirteen children with alpha 1-antitrypsin deficiency (8 PiZ and 5 PiSZ) were investigated at ages ranging from 4 to 6. None had had neonatal cholestasis. Nine, mainly the PiZ individuals, had increased serum concentration of transaminases. Liver biopsy was performed in 7 patients with increased serum levels of transaminases. One of these patients had cirrhosis and 4 had moderate to severe fibrosis. The results indicate that alpha 1-antitrypsin deficient individuals, also without neonatal cholestasis syndrome run a high risk of developing serious liver disease, already in childhood. The cirrhotic patient was the only one who had increased excretion of bile acids in urine.
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PMID:Liver disease in children with alpha 1-antitrypsin deficiency without neonatal cholestasis. 698 76

Ten children with alpha 1-antitrypsin deficiency, phenotype PiZ, and neonatal cholestasis were followed to the ages of 4-20 years. Standard liver function tests, urinary bile acid excretion and liver morphology were investigated within one week and the results were compared. Three patients had morphological signs of liver cirrhosis and one of these had clinical signs as well. Compared to healthy children of the same age urinary bile acid excretion was increased only in those with morphologically confirmed liver cirrhosis. Although most of the other patients had some minor histological changes in the liver biopsies, the normal urinary bile acid excretion was in accordance with their excellent general clinical state and is interpreted as a short-term favourable prognostic sign.
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PMID:Natural history of children with alpha 1-antitrypsin deficiency and neonatal cholestasis. 698 78

Serum bile acids were studied in 34 patients, aged 7/12 to 20 years, with alpha 1-antitrypsin deficiency. Of these patients, 27 were of Pi-phenotype Z and 7 of SZ. Liver biopsy according to Menghini was performed in 19 patients. Fasting serum levels of cholic and chenodeoxycholic acids, determined by radioimmunoassay, were compared to conventional liver function tests and liver morphology. All patients with morphological liver cirrhosis had increased fasting levels of serum bile acids. The other patients, including those with severe fibrosis, had normal values. Although standard liver function tests were more pathological in the cirrhotic patients than in the others, serum fasting bile acids seemed to be the most distinct markers of severe liver disease.
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PMID:Serum bile acids as markers of juvenile liver disease in alpha 1-antitrypsin deficiency. 698 62

Blood coagulation and fibrinolysis before and after splenectomy was studied in 74 cases of liver cirrhosis. A hypocoagulable state was found before splenectomy, but the platelet count, and the levels of fibrinogen, plasminogen, alpha 2-macroglobulin and antithrombin III increased significantly after splenectomy (p less than 0.05 to p less than 0.001). A marked improvement was observed on the values of r (reaction time), k (clot formation time) and ma (maximal amplitude) of thrombelastograms (p less than 0.05 to p less than 0.001). The prothrombin time was reduced after the surgery, but not significantly (p less than 0.1, greater than 0.05). The levels of alpha 1-antitrypsin remained almost unchanged, while serum fibrinogen-fibrin degradation products (FDP) showed a slight decrease postoperatively. The immunohistologic study of the spleen excised from 7 cases with liver cirrhosis, with the use of the direct immunofluorescence technique, demonstrated the deposits of fibrin in the splenic cords in all cases. It was not recognized in the spleens of 4 cases without cirrhosis used as the control. A further study of the spleen weight and plasma fibrinogen level showed that a significant inverse correlation exists between these two parameters (p less than 0.01). These findings suggest that localized intravascular coagulation (LIC) occurs in the enlarged spleen associated with liver cirrhosis.
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PMID:Effects of splenectomy on blood coagulation and fibrinolysis in patients with liver cirrhosis: possible role of the spleen in haemostasis. 698 11

The term "chronic hepatitis" includes diseases of different etiology, i.e. viral (hepatitis B virus, non-A/non-B hepatitis virus[es]), drug-induced (e.g. oxyphenisatin, alpha-methyldopa), metabolic (Wilson's disease, alpha 1-antitrypsin deficiency) and so-called "autoimmune" hepatitis. In the clinical course, hepatitis running for up to 3 months is considered acute; when lasting for 3-6 months it is termed prolonged, and chronic hepatitis means a duration of more than 6 months by definition. In chronic hepatitis there is international agreement on basing nomenclature on morphologic findings and on distinguishing chronic persistent hepatitis (with a predominantly lymphocytic inflammation restricted to portal tracts) from chronic aggressive (or active) hepatitis. The latter is typified by piecemeal necrosis in the periportal areas (activity a); additional piecemeal necrosis along fibrous septa or bridging hepatic necorsis is the key feature for activity b. In hepatitis B virus infection, the symptomfree carrier of the virus with no inflammation in biopsy, or merely nonspecific reactive hepatitis, must be included under the heading of chronic hepatitis. Cirrhosis, however, although resulting from necrosis, inflammation and fiber formation, refers exclusively to the disturbance of lobular architecture and its microcirculatory consequences. The term "cirrhosis" is thus not included in the definition of chrome hepatitis and should be evaluated separately as an entity in its own right.
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PMID:[Chronic hepatitis]. 742 73

Hepatocellular carcinoma (HCC) is among the 10 most common tumors in the world. However, incidence is not evenly distributed across the world. In many instances, the proximate cause for the tumor can be identified. Chronic hepatitis B infection is probably the most common cause, followed by chronic hepatitis C. Other important causes are alcoholic liver disease, hemochromatosis, alpha 1-antitrypsin deficiency, and other chronic liver diseases. Although proximate causes may be identifiable, pathogenesis remains uncertain. Factors that may be important include the presence of Aflatoxin B1 in food, genetic changes induced by the hepatitis B virus, and repeated rounds of necrosis and regeneration, also induced by hepatitis viruses. The genes involved and the mutations necessary for hepatic carcinogenesis are unknown, with the sole exception of the p53 gene, which is probably a late phenomenon. Screening for HCC is widely practiced despite the lack of evidence of improved survival. The screening tests used include alphafetoprotein levels and ultrasonography. Screening can identify small tumors; however, survival may not be improved, because the presence of cirrhosis may limit the number of patients who can undergo resections; recurrences or second primary tumors are common; and the presence of chronic liver disease means that survival may be limited anyway. There are many different forms of therapy available; unfortunately, most have not been compared in randomized controlled trials. Surgery remains the therapy of choice if feasible. All other therapy is palliative, including chemotherapy, chemoembolization, hepatic artery embolization, various forms of radiotherapy, and various forms of ablative therapy.
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PMID:Hepatocellular carcinoma. 753 16

We report a case of cirrhosis developing in a man who was heterozygous for alpha 1-antitrypsin deficiency and who was receiving methotrexate for severe rheumatoid arthritis. The alpha 1-antitrypsin phenotype PiMZ has been associated with cryptogenic cirrhosis. Our patient had no biochemical or histologic evidence of chronic liver disease during the first year of receiving methotrexate. We postulate that the PiMZ state may result in enhanced susceptibility to methotrexate-induced hepatic toxicity and should be screened for if liver function abnormalities occur during methotrexate therapy.
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PMID:End-stage liver disease developing with the use of methotrexate in heterozygous alpha 1-antitrypsin deficiency. 761 33

Of 200,000 Swedish infants screened for alpha 1-antitrypsin deficiency (alpha 1 ATD), 184 (127 PiZ, 2 PiZ-, 54 PiSZ, and 1 PiS-) children have been followed prospectively, of whom 1 PiSZ and 5 PiZ children died in early childhood. We now report clinical and biochemical signs of liver disease in adolescence and the prognosis of neonatal liver disease up to the age of 18 years. The alpha 1 ATD subjects were offered a clinical checkup and liver tests at 16 and 18 years of age, 150 of 178 alpha 1ATD subjects undergoing checkups at age 16 and 166 at age 18. Liver tests were performed in 121 adolescents at both the 16- and 18-year checkups. None of the PiZ and PiSZ subjects checked at the age of 16 and 18 years had any clinical signs of liver disease. Abnormalities of serum alanine aminotransferase (S-ALAT) or gamma-glutamyl transferase (S-GT) were found at the 16-year checkup (all PiZ and PiSZ subjects tested included) in 17% of PiZ and 8% of PiSZ adolescents, and at the age of 18 years in 12% of PiZ and 15% of PiSZ subjects. In only two cases were both S-ALAT and S-GT concentrations abnormal at both the 16-year and 18-year follow-ups. Serum procollagen III peptide concentrations were normal in all those with abnormal liver test results. Of 127 PiZ subjects, 22 had manifested clinical signs of liver disease in infancy. Of these 22, two died early in life of cirrhosis.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:The liver in adolescents with alpha 1-antitrypsin deficiency. 763 38

The aim of the present was to define prognosis and life expectancy in patients with chronic liver disease of different etiologies and to relate them to an age- and sex-matched normal population. After a follow-up of 15 years, life expectancy of 620 patients with chronic liver disease was retrospectively calculated and compared with an age- and sex-matched normal population. Among patients with cirrhosis, prognosis was dependent upon Child classification (P = 0.001). Patients with alcoholic cirrhosis and fatty liver disease were younger (P = 0.01) and had a lower life expectancy than patients with other causes of chronic liver disease (P = 0.004). Patients with hepatitis B and hepatitis C cirrhosis showed a comparable prognosis and a significantly lower life expectancy than the age- and sex-matched population. Cryptogenic and autoimmune liver diseases showed a comparable life expectancy but a significantly shorter life expectancy than the normal population. In patients with alpha 1-antitrypsin deficiency-associated cirrhosis, a high viral coinfection rate was found (P = 0.01). For patients with noncirrhotic hemochromatosis, prognosis was poorer than that for the age- and sex-matched population. In patients with asymptomatic primary biliary cirrhosis, chronic persistent hepatitis B, and alpha 1-antitrypsin deficiency without cirrhosis, life expectancy was equal to that of the normal population. Prognosis and life expectancy in chronic liver disease depend on stage, cause, and symptoms of chronic liver disease; age; and possibilities of treatment. In patients with hereditary liver disease, additional viral infection of alcohol abuse lead to a significant deterioration of life expectancy. Patients with alcoholic chronic liver disease have the poorest prognosis.
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PMID:Prognosis and life expectancy in chronic liver disease. 764 84

The aim of the present study was to determine the prevalence of hepatocellular carcinoma in adults with heterozygous alpha 1-antitrypsin deficiency and to assess the presence of possible co-risk factors for the development of hepatocellular carcinoma. Two hundred and forty patients with cirrhosis of different aetiologies and 130 patients with alpha 1-antitrypsin deficiency without evidence of chronic liver disease were investigated. Out of the 240 patients with cirrhosis, 61 patients (25%) were found to have alpha 1-antitrypsin deficiency, 36 patients (15%) had chronic hepatitis C infection, 50 (21%) had chronic hepatitis B and 24 (10%) had hepatitis C and hepatitis B infection. Thirty patients (12%) had cryptogenic cirrhosis and 39 (16%) alcoholic cirrhosis. The prevalence of hepatocellular carcinoma in patients with alpha 1-antitrypsin deficiency-associated cirrhosis was comparable to that of hepatocellular carcinoma in patients with cirrhosis of other aetiologies. Positive viral markers were found in 67% of the patients with alpha 1-antitrypsin deficiency-associated cirrhosis with hepatocellular carcinoma. In contrast, in the group of 130 patients with alpha 1-antitrypsin deficiency but without clinical and laboratory signs of chronic liver disease, none was found to have hepatocellular carcinoma (p = 0.001). Our results indicate that heterozygous alpha 1-antitrypsin deficiency-associated cirrhosis is a risk factor for hepatocellular carcinoma, but this is due to chronic liver disease and not due to the metabolic disorder itself.
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PMID:Prevalence of hepatocellular carcinoma in alpha-1-antitrypsin deficiency. 769 20


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