Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

alpha 1 antitrypsin deficiency is associated with predisposition to the development of pulmonary emphysema and childhood cirrhosis. There are two common deficiency alleles in the European population, proteinase inhibitor (Pi) Z and S. In addition, there are rare Pinull or QO variants which can be difficult to diagnose. A family assigned as having the PiQO allele by AAT protein quantification and isoelectric focusing was shown by DNA sequencing to have the PiMheerlen mutation (Pro369-Leu). This highlights the difficulties of diagnosis of PiQO.
 ...
PMID:What is Pi (proteinase inhibitor) null or PiQO?: a problem highlighted by the alpha 1 antitrypsin Mheerlen mutation. 155 39

Researchers analyzed 1983 vital statistics for Taiwan using the life table analysis and calculating the potential years of life lost (PYLL) to identify life shortening features of several major causes of death and examine the preventability of premature mortality. Life expectancy for males was 69.9 years whereas for females it was 75.1 years. these corresponding life expectancies for Japan were 73.8 years and 79.6 years and 71.7 years and 78.7 years for the US. Between 1950-1983, life expectancy at birth in Taiwan increased 17.5 years for males and 19 years for females. In 1983, the cumulative total of PYLL before age 70 for males was 737,205 compared to 353,780 years for females. Thus loss of productivity of males was 2 times that of females. Most of this loss was a result of accidents. In fact, they contributed to 37.2% of PYLL for males and 24.9% of PYLL for females. Even though accidents were also the leading cause for the most PYLL in Japan and the US (17.4% and 8.7% respectively in Japan and 25% for both sexes combined in the US), the magnitude was considerably lower than that of Taiwan. Further the type of accidents males were more likely to die from were motor vehicle accidents (18,8% vs. 18.4% for home and workplace accidents). On the other hand, women were more likely to die from home and workplace accidents(14% vs. 10%). The 2nd major cause for the most PYLL was all cancers, except liver cancer, (15.9% for males and 20.4% for females). The 3rd major cause for the most PYLL for males was liver disease (liver cancer and cirrhosis of the liver) (9.6%) while for females it was stroke (8.7%). Further suicides contributed to 6.5% of PYLL for females. IN conclusion, Taiwan should place accident prevention as a high priority since it needs limited resources and is more achievable than that of cancer elimination. The next level of preventive efforts should include stroke and suicide.
Asia Pac J Public Health 1991
PMID:Mortality trend in a rapidly developing economy in Taiwan. Part II: Life expectancy and "potential years of life lost". 179 32

A total of 70 serum samples taken from patients with hepatocellular carcinoma (HCC), acute viral hepatitis and cirrhosis and normal individuals were tested in a binding inhibition immunofluorescence assay using 5 mouse monoclonal antibodies (2G9, 3H11, 3H12, 1C7, 3H5) specific for hepatoma cells. Seven out of the 30 HCC sera (23.3%) inhibited the binding of one of these antibodies, 3H11. This detection of antigen 3H11 or antigens of similar structure in HCC sera was significantly more frequent than in control sera (1/40 = 2.5%) (Fisher's exact test, p = 0.009; df = 1, relative risk calculated by the odds ratio in a 2 x 2 table = 12.0). The presence of this antigen was unrelated to the hepatitis B surface antigen and alphafetoprotein status. Thus it would be of value particularly for the detection of hepatitis B negative or alphafetoprotein negative liver cancers.
Asian Pac J Allergy Immunol 1990 Dec
PMID:Detection of tumour antigens in sera of patients with hepatocellular carcinoma using monoclonal antibodies. 196 8

The authors investigated dynamic changes and the interaction of the plasma renin activity (PRA), plasma aldosterone (PAC), i.e. the main representatives of sodium retaining systems, and of the atrial natriuretic factor (ANF) the decisive natriuretic substance in acute expansion of the extracellular volume (ECV) by infusion of two litres of saline in six controls, seven patients with essential hypertension and liver cirrhosis without ascites (6 patients) and with ascites (6 patients). The expansion evoked controversial changes of these systems. It led to a rise of ANF and suppression of PAC and PRA. Although ANF rose after infusion to the roughly similar range (12.4 to 15.7 pmol/l), the natriuretic response to expansion differed significantly in different groups of patients. It was most marked in hypertonic subjects (517.2 to 93.2 mumols/min) and practically zero in ascitic liver cirrhosis (54.2 +/- 44.2 mumols/min). The explanation of this finding may be the persistence of high activity of the renin-angiotensin-aldosterone system despite its partial inhibition by infusion of saline in cirrhosis of the liver (PRA 1.69 +/- 0.66 nmols/l/hr., PAC 1.12 nmol/l). For the renal response to acute expansion of the ECV thus not only the absolute plasma concentration of ANF is decisive but also its ratio to the activity of the sodium retaining renin-angiotensin-aldosterone system.
 ...
PMID:[Mutual interaction of the renin-angiotensin system and the atrial natriuretic factor in the renal response to acute volume loading]. 214 4

Ascitic fluid alpha 1-antitrypsin (AF-AAT) was compared with ascitic fluid total protein (AF-TP) and the serum-ascites albumin gradient (SAAG) in the differential diagnosis of ascites. The study included 82 consecutive patients of which 42 had cirrhosis, 8 hepatoma (with cirrhosis), and 27 malignant ascites (peritoneal 18, liver 9). The concentration of AF-AAT (milligrams per deciliter) was significantly elevated (P less than 0.001) in hepatoma (174 +/- 123), malignant liver disease (232 +/- 119) and peritoneal neoplasms (376 +/- 106) in comparison with cirrhotics (66 +/- 33). In separating ascites caused by cirrhosis or malignancy, AF-AAT (discriminating limit of 120 mg/dl) had a 96% sensitivity, 95% specificity, and 96% diagnostic efficacy, which was superior to the 87% observed for AF-TP and 86% for the SAAG. Similar results were obtained for the A/S AAT ratio but this test was not available in all patients. AF-AAT was particularly useful in patients with malignancy causing portal hypertension as assessed by SAAG (hepatoma, malignant liver disease). We conclude that AF-AAT may be a valuable parameter in the differential diagnosis of ascites.
 ...
PMID:Ascitic fluid alpha 1-antitrypsin. 216 27

The presence and distribution of AFP, AAT and HBsAg in peritumoral non-neoplastic hepatocytes (NNH) of 27 cases and, at the same time, in the neoplastic tissue of 37 liver cell carcinoma (HCC) were studied; AFP and HBsAg were more frequently found in NNH than in HCC cells; no differences were found for AAT. The presence of HBsAg also in normal liver without cirrhosis is probably best explained by its possible role in neoplastic transformation and by the inhibition of replication of the viruses AFP, considered to be expression of dedifferentiated cells, may possible be taken up by NNH for catabolic purposes.
 ...
PMID:Immunohistochemical study of the appearance of some markers in liver adjoining hepatocellular carcinoma. 242 60

Primary liver carcinoma (PLC) may express a certain number of markers. Here we communicate results of an analysis of five such markers (alpha-1-antitrypsin--AAT--, carcino-embryonic antigen --CEA--, alpha-fetoprotein --AFP--, and superficial --HBsAg-- and core --HBcAg-- antigens of hepatitis B virus) by means of PAP techniques in 130 cases of PLC, comparing the neoplastic tissue and the non-tumorous liver. Three variants of PLC are distinguished: hepatocarcinoma (HC) (108 cases); cholangiocarcinoma (CC) (19 cases); and three cases of hepatocholangiocarcinoma (HCC). AAT was positive in 29 HC, 2 HCC, and negative in all 19 CC. CEA appeared positive in 16 HC, 16 CC and only one HCC. AFP was positive in two HC, and negative in all CC and HCC. HBsAg displayed positivity in 15 HC and one HCC, being negative in all 19 CC. HBcAg was positive in 4 HC, and negative in all CC and HCC. HBsAg was also positive in two neoplastic emboli associated with HC. On the non-tumorous liver tissue the immunohistochemical results showed positivity for AAT and CEA, but not for AFP. Therefore the present results confirm that in the geographical area from which these tumors proceed, PLC is closely correlated with HBsAg positivity and with cirrhosis.
 ...
PMID:Immunohistochemical characterization of 130 cases of primary hepatic carcinomas. 244 80

In 500 consecutive autopsies there were 27 cases in which the livers contained PAS-positive, diastase-resistant globules within hepatocytes. On the basis of morphologic findings and immunoperoxidase staining the inclusions were separable into two groups. There were 14 (2.8%) cases in which the globules were periportal in location and stained positively with the specific AAT immunoperoxidase method (Type 1 globules). In 13 (2.6%) cases, the globules were located in the centrilobular region of the liver or at the edge of the central ischemic zone. These globules did not stain with the specific immunoperoxidase technic (Type 2 globules). Cirrhosis was found in 10 (71%) of the 14 livers containing Type 1 globules. Dysplastic liver cells were present in four cases. No liver cell cancer was present in any of the cases. No fibrosis or cirrhosis was found in any of the 13 livers containing Type 2 globules. They were always present in the centrilobular areas and most likely were the result of sinusoidal congestion and anoxia. The immunocytochemical method is useful in separating the two types of PAS-positive, diastase-resistant globules. Type 1 inclusions are associated with alpha 1 antitrypsin deficiency.
 ...
PMID:Alpha 1 antitrypsin liver disease differential diagnosis of PAS-positive, diastase-resistant globules in liver cells. 618 89

Alpha 1-antitrypsin has been examined in formalin-fixed, paraffin-embedded liver specimens from Greek patients with cirrhosis (35 cases) and hepatoma (55 cases) by peroxidase-antiperoxidase (PAP) method. Ring-like AAT globules were found in the non-neoplastic cells in 12% of the cases of hepatoma and in 11% of the cases of cirrhosis. Atypical globules were seen in neoplastic cells in 5.4% of the cases of hepatoma and in 17% of the cases of liver cirrhosis. A diffuse fine granular pattern of AAT distribution was present in 31% of the cases of hepatoma in the neoplastic cells and in 27% of those in the non-neoplastic cells. The relatively high incidence of ring-like AAT-globules, and of atypical globules in cases of hepatoma and cirrhosis is not in agreement with the extremely low gene frequency of Z allele in a Greek population of patients with cirrhosis and hepatoma. Thus, there is some doubt whether AAT-globules in the liver represent a histopathologic marker of genetically determined AAT deficiency. A relationship between AAT deposits and the degree of differentiation of hepatoma was noted in this series. AAT-positive cells were found in 55% of moderately differentiated, in 29% of highly differentiated and in 20% of poorly differentiated hepatomas.
 ...
PMID:Demonstration of alpha 1-antitrypsin in paraffin sections of hepatoma and cirrhosis. 629 75

A 10.5 year old girl with liver cirrhosis due to AAT-deficiency (Pi type ZZ) developed cyanosis and clubbing of finger and toes. Clinical aspect of a cyanotic heart disease appeared with 10 years, 7 years after diagnosis of cirrhosis. By contrast echocardiography existence of intrapulmonary arterio-venous shunts was demonstrated. When determined during the first year of life, serum-alpha-1-globulin-fraction of the patient was found to be normal. The result indicates, that even in severe AAT-deficiency of Pi type ZZ direct determination of AAT is necessary for diagnosis of the disease.
 ...
PMID:[Liver cirrhosis due in alpha-1-antitrypsinin deficiency and development of an arteriovenous shunts of the lungs]. 660 21


1 2 3 4 5 6 7 8 Next >>