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)

Hepatic phosphorus-31 magnetic resonance spectroscopy (31P MRS) was undertaken in 85 patients with histologically proven cirrhosis of varying etiologies and functional severity. Reference data were acquired from 16 healthy volunteers who had no history or evidence of liver disease or alcohol abuse. In vivo hepatic 31P MR spectra were acquired with pulse angle 45 degrees and repetition times (TR) of 5 and 0.5 seconds. Peak area ratios of phosphomonoesters (PME), inorganic phosphate (Pi), and phosphodiesters (PDE) relative to beta ATP, and of PME relative to PDE were calculated from spectra acquired at TR 5 seconds. Estimates of saturation effects for individual resonances were obtained by dividing the peak height at TR 5 seconds by that at TR 0.5 seconds to yield a T1-related signal height ratio (SHR). When compared with reference values, the patients with liver disease showed a significantly higher PME/ATP (P < .0001), PME/PDE (P < .0001), PME SHR (P < .001), and Pi SHR (P < .02), and a lower PDE/ATP (P < .001) and PDE SHR (P < .001). The magnitude of these changes increased significantly and progressively with increasing functional impairment. In patients with compensated cirrhosis spectral appearances varied with etiology; thus, patients with postviral cirrhosis showed a significantly higher Pi/ATP; those with alcoholic cirrhosis, a significantly lower PDE/ATP; and those with cirrhosis secondary to primary sclerosing cholangitis, a significantly lower Pi/ATP than the healthy volunteers or other etiological groups. However, spectral appearances did not vary with etiology in patients with decompensated disease. In vitro 31P MRS of perchloric extracts of samples of liver tissue obtained from 10 patients with cirrhosis at transplant hepatectomy showed increases in levels of the soluble PME metabolites, phosphorylcholine and phosphorylethanolamine, and reductions in the levels of the soluble PDE metabolites, glycerophosphorylcholine and glycerophosphorylethanolamine. These changes suggest regenerative activity in cirrhotic livers. The increases in soluble phosphomonoesters in the aqueous extracts accounted for the increased PME/ATP ratio seen in the in vivo spectra, and might account for the increase in PME SHR. The reduction in soluble phosphodiesters in the aqueous extracts did not entirely account for the reduction PDE/ATP ratio seen in vivo.(ABSTRACT TRUNCATED AT 400 WORDS)
 ...
PMID:Effect of functional grade and etiology on in vivo hepatic phosphorus-31 magnetic resonance spectroscopy in cirrhosis: biochemical basis of spectral appearances. 784 15

9 cases (males) of primary sclerosing cholangitis (PSC) were studied clinico-morphologically. In puncture liver biopsies, sclerosis of walls of big ducts and portal tracts was found in 2 cases only; cell infiltration was minimal. Lobular hepatitis with intralobular fibrosis and moderate sclerosis of portal stroma was found in 3 patients, monolobular inactive liver cirrhosis in 4 patients. In all cases mild intralobular cholestasis was found which was followed with a considerable increase of copper and manganese in the liver and serum. The accumulation of a large amount of collagen of different types was observed around bile ducts which ultrastructurally was seen as fibrillar structures and homogeneous substance similar to the basal membrane. The elements of the epithelium destruction were found in foci of the duct basal membrane alteration, in the absence of cell infiltration. Progressing fibrosis of both portal tracts without inflammation and individual segments of the bile ducts and the intralobular stroma is at the basis of PSC.
 ...
PMID:[Morphological changes in the liver in primary sclerosing cholangitis (a histological and electron microscopic study)]. 794 63

In previous studies, the majority of patients with the cholestatic liver diseases, primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC), had increased hepatic copper (Cu) levels even in early stages of disease. We prospectively measured hepatic copper content by atomic absorption spectrophotometry in 55 patients with PBC, 6 patients with PSC, and 29 patients with other chronic noncholestatic liver diseases. Hepatic Cu content was normal in 22/61 (36%) of patients with PBC or PSC; 18 of the 22 did not have cirrhosis (82%). Hepatic Cu content increased with increasing stage of disease (r = 0.61, P < 0.001) and was positively correlated with serum total bilirubin (r = 0.6, P < 0.0001) and alkaline phosphatase (r = 0.5, P < 0.001). All patients with stage I and II disease had hepatic Cu < 150 micrograms/g dry weight, and all patients with hepatic Cu > 150 micrograms/g dry weight had stage III and IV disease. Hepatic Cu content is normal in early PBC and PSC. Copper accumulation in the liver in these cholestatic liver diseases is secondary to cholestasis rather than a primary phenomenon.
 ...
PMID:Hepatic copper content is normal in early primary biliary cirrhosis and primary sclerosing cholangitis. 795 10

The expression of Lewis Y antigen (a blood group-related antigen containing type 2 chain) in hepatocytes was examined immunohistochemically, using the monoclonal antibody F-3, in a total of 530 liver specimens consisting of normal livers and various hepatobiliary diseased livers. Lewis Y was rarely expressed in the hepatocytes in histologically normal livers (20 cases) or in livers from patients with nonspecific reactive change or chronic persistent hepatitis, in viral livers (19 cases), autoimmune etiology livers (3 cases), or acute hepatitis-like change of autoimmune etiology livers (7 cases). In chronic active hepatitis of viral (132 cases) and autoimmune etiology livers (20 cases) with or without cirrhosis, the incidence of membranous expression of Lewis Y in clustered hepatocytes of viral and autoimmune etiology livers was 21 and 0% of mild chronic active hepatitis; 69 and 25% of moderate degree; 85 and 50% of severe degree; and 87 and 100% of cirrhosis, respectively. In alcoholic liver disease (27 cases), the incidence of membranous expression of Lewis Y in clustered hepatocytes was 18, 75, and 100% in mild fibrosis, moderate fibrosis, and cirrhosis, respectively. Such membranous expression of Lewis Y was not found in the early or late stages of the biliary diseases examined, primary biliary cirrhosis (226 cases), primary sclerosing cholangitis (8 cases), or extrahepatic biliary obstruction (22 cases).(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:Membranous expression of Lewis Y antigen in clustered hepatocytes in chronic viral, autoimmune, and alcoholic liver diseases but not in biliary diseases. 805 6

The peribiliary vascular plexus (PVP) plays an important role in the pathophysiology of the biliary tree. We histologically examined vascular endothelial cells of the intrahepatic PVP in various hepatobiliary diseases by immunohistochemistry and lectin histochemistry with antibodies to factor VIII-related antigens (F-VIII-R-Ag) and Ulex europaeus agglutinin I (UEA-I). The PVP around the intrahepatic large bile ducts (LBDs) and septal bile ducts (SBDs) in normal livers consists of three layers: inner layer vessels immediately adjacent to the epithelium, intermediate layer vessels within the ductal wall, and outer layer vessels outside the ductal wall. In some bile ducts that show active inflammation in hepatolithiasis, primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC), and extrahepatic biliary obstruction (EBO), vessels in the intermediate layer and, to a lesser degree, in the inner layer, are increased in number. In sclerotic bile ducts of PSC, EBO, and hepatolithiasis, the number of inner and intermediate layer vessels are markedly and variably reduced, respectively. In liver cirrhosis or chronic advanced liver diseases, the vessels in all three layers, particularly those in the outer layer, are increased in number and dilated, probably reflecting intrahepatic microcirculatory disturbance. The PVP showed several types of numerical and luminal changes, each of which may be related to disease processes in the intrahepatic biliary tree as well as to intrahepatic microcirculatory disturbance.
 ...
PMID:Intrahepatic peribiliary vascular plexus in various hepatobiliary diseases: a histological survey. 808 71

Of 10 patients with ulcerative colitis 2 had primary sclerosing cholangitis and 1 had cirrhosis due to chronic active hepatitis. All 3 underwent successful orthotopic liver transplantation due to severe portal hypertension and deteriorating liver function. The interrelations between liver transplantation, immunosuppressive therapy and bowel disease are described.
 ...
PMID:[Interrelation between liver and bowel involvement in ulcerative colitis patients undergoing liver transplantation]. 811 62

Controlled trials to assess the therapeutic benefit of orthotopic hepatic transplantation (OHTx) for primary sclerosing cholangitis (PSC) cannot be justified in view of improvement of patient survival after this operation since 1981. However, the actual patient survival with OHTx can be compared with the Mayo model estimated survival probabilities without OHTx. This model, which encompasses physical, biochemical and histopathologic parameters of PSC, was constructed from a study of 392 conservatively treated PSC patients at five international centers in England and North America. We compared the actual survival of 216 adult patients with the diagnosis of advanced PSC who underwent hepatic replacement with the expected survival estimated by the Mayo PSC natural history model, "the simulated control technique." OHTx was performed at the University of Pittsburgh and Mayo Medical Center between 5 December 1981 and 26 December 1990. The mean (plus or minus standard deviation) post-OHTx follow-up period was 34 +/- 25 months (range of zero to 104 months). Before transplantation, biliary or portal hypertensive operation, or both, was performed upon 104 patients. At operation, the mean age of recipients was 42.1 +/- 11.3 years and the mean value of total serum bilirubin was 13.3 +/- 13.0 milligrams per deciliter. Extensive septal fibrosis and cirrhosis were histologically documented in 97 percent of the patients, with splenomegaly in 63 percent. Immunosuppressive therapy was based primarily on cyclosporin in 184 recipients and FK-506 in 32. Within six months, the Kaplan-Meier survival probability after OHTx (0.89) already was higher than predicted by the Mayo model (0.83). At five years, the Kaplan-Meier actual survival with OHTx was 0.73 compared with 0.28 expected Mayo model survival. The overall increased survival rate with transplantation was statistically significant (chi-square equals 126.6; p < 0.001). At all risk stratifications, OHTx significantly improved survival with a p value of 0.031 (low risk), 0.001 (moderate risk) and < 0.001 (high risk). Thus, OHTx is effective therapy for PSC. Disease gravity and unsuspected cholangiocarcinoma in the excised native liver adversely influenced short and long term survival rates after transplantation, respectively.
 ...
PMID:Efficacy of hepatic transplantation in patients with primary sclerosing cholangitis. 821 75

Analysis of 5180 liver transplant cases from 37 liver transplant centers in the United States (1982-1991) shows an overall one-year survival rate of 79.4 +/- 0.6% and a five-year survival rate of 69.2 +/- 0.9%. There was marked improvement in the one-year survival rate after liver transplantation from 36.0 +/- 9.6% in 1982 to 85.0 +/- 1.8% in 1991. One-year survival rates after liver transplantation for postnecrotic cirrhosis, primary biliary cirrhosis, alcoholic cirrhosis, primary sclerosing cholangitis, alpha-1-antitrypsin deficiency, and Wilson's disease ranged from 78.4 +/- 1.0% to 84.2 +/- 1.5% and five-year survival rates from 68.6 +/- 3.8% to 79.2 +/- 5.3%. Survival rates after liver transplantation for hemochromatosis were poor--a one-year survival rate of 53.8 +/- 6.8% and a five year survival rate of 43.1 +/- 11%. One- and five-year survival rates for the 0-13 years age group were 74.6 +/- 2.8% and 66.7 +/- 3.4%; for the 14-37 years age group, 83.3 +/- 1.2% and 73.8 +/- 1.8%; for the 38-54 years age group, 79.6 +/- 0.8% and 69.7 +/- 1.3%; for the 55-63 years age group, 76.0 +/- 1.4% and 63.0 +/- 3.1%; and for the 64-77 years age group, 76.5 +/- 3.0% and 65.4 +/- 4.6%.
 ...
PMID:An analysis of liver transplant experience from 37 transplant centers as reported to Medicare. 821 49

Three patients with primary sclerosing cholangitis and Hodgkin's disease, a previously unrecognized association, are reported. All three patients were men, and one patient had Crohn's disease of the colon. Primary sclerosing cholangitis was diagnosed 2, 11 and 17 yr before diagnosis of Hodgkin's disease in the three patients, and all three had advanced biliary cirrhosis prompting referral for liver transplantation. The symptoms of Hodgkin's disease were often masked by similar manifestations of primary sclerosing cholangitis, particularly symptoms of recurrent biliary sepsis. Hodgkin's disease is another disorder that may occur in patients with primary sclerosing cholangitis, particularly in the setting of advanced disease, and may be masked by the underlying hepatobiliary disease.
 ...
PMID:Primary sclerosing cholangitis and Hodgkin's disease. 822 18

The patterns of hepatic injury were studied in 100 patients with a diagnosis of sarcoidosis and clinical evidence of liver disease that led to diagnostic liver biopsy. Granulomas were present in all patients; they occupied from < 1% to > 90% of the total volume of tissue examined and were most often located in the portal/periportal region. In none of the 100 cases were infectious organisms identified by special stains, culture, or serology. In 99% of cases, these granulomas were noncaseating; in one of the 100 cases central caseation was noted. In addition to the granulomas present in all biopsies, three broad categories of histologic change were found: cholestatic (58%), necroinflammatory (41%), and vascular (20%). Among those with cholestasis, 19 patients had bile duct lesions similar to primary biliary cirrhosis, whereas another 13 had a pattern of periductal fibrosis reminiscent of primary sclerosing cholangitis. In 37 patients with chronic cholestasis, a decrease in the number of bile ducts (ductopenia) was noted. Twelve patients had an acute cholangitis suggestive of mechanical obstruction--although no clinical evidence of ductal obstruction was found. Necroinflammatory changes included spotty necrosis suggesting hepatitis of diverse etiologies (including viral infection and drug reaction) and chronic portal inflammation suggestive of chronic active hepatitis. Vascular changes consisted of sinusoidal dilatation (14 cases) and nodular regenerative hyperplasia (9 cases). In 6% of the patients, the only changes in the biopsy were those of granulomatous inflammation; each of these patients had a dominant mass ("sarcoidoma"), which had been biopsied to rule out tumor. Fibrosis was seen in 21% of the biopsies--periportal (13%), bridging (2%), or cirrhosis (6%). It is clear that sarcoidosis can cause progressive liver disease with a wide array of histologic features that can mimic those of other primary liver diseases.
 ...
PMID:Hepatic sarcoidosis. Clinicopathologic features in 100 patients. 823 35


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>