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An automated microassay for enzyme inhibitory effects of M2 antibodies in primary biliary cirrhosis. 原发性胆汁性肝硬化中M2抗体酶抑制作用的自动微量测定。
Liver Pub Date : 1991-10-01 DOI: 10.1111/j.1600-0676.1991.tb00531.x
K L Teoh, M J Rowley, I R Mackay
{"title":"An automated microassay for enzyme inhibitory effects of M2 antibodies in primary biliary cirrhosis.","authors":"K L Teoh,&nbsp;M J Rowley,&nbsp;I R Mackay","doi":"10.1111/j.1600-0676.1991.tb00531.x","DOIUrl":"https://doi.org/10.1111/j.1600-0676.1991.tb00531.x","url":null,"abstract":"<p><p>In primary biliary cirrhosis (PBC), autoantibodies are produced to the M2 group of mitochondrial antigens, of which a major constituent is the E2 subunit of the pyruvate dehydrogenase complex (PDC). These antibodies, in addition to conventional reactivities with PDC, characteristically inhibit the catalytic function of PDC in vitro, as judged by a macroinhibition assay based on spectrophotometry. We describe a microinhibition assay adapted for microtitre plates and for an automated readout of results by an ELISA plate reader. We show that this microassay has a sensitivity similar to that of the macroassay. In a study of 83 sera, from PBC and other diseases, the inhibitory microassay proved specific for PBC. This automated inhibitory microassay for PBC-sera could become a primary laboratory procedure for the diagnosis of PBC, particularly because it may identify antibodies to the actual autoepitope on the PDC-E2.</p>","PeriodicalId":18183,"journal":{"name":"Liver","volume":"11 5","pages":"287-91"},"PeriodicalIF":0.0,"publicationDate":"1991-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1111/j.1600-0676.1991.tb00531.x","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"12884771","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 17
Ultrastructural localization of type IV collagen and laminin in the Disse space of rat liver with carbon tetrachloride induced fibrosis. 四氯化碳肝纤维化大鼠肝间隙内IV型胶原和层粘连蛋白的超微结构定位。
Liver Pub Date : 1991-10-01 DOI: 10.1111/j.1600-0676.1991.tb00528.x
Y Nakayama, T Takahara, C R Miyabayashi, H Itoh, A Watanabe, H Sasaki, Y Muragaki, A Ooshima, K Inoue
{"title":"Ultrastructural localization of type IV collagen and laminin in the Disse space of rat liver with carbon tetrachloride induced fibrosis.","authors":"Y Nakayama,&nbsp;T Takahara,&nbsp;C R Miyabayashi,&nbsp;H Itoh,&nbsp;A Watanabe,&nbsp;H Sasaki,&nbsp;Y Muragaki,&nbsp;A Ooshima,&nbsp;K Inoue","doi":"10.1111/j.1600-0676.1991.tb00528.x","DOIUrl":"https://doi.org/10.1111/j.1600-0676.1991.tb00528.x","url":null,"abstract":"<p><p>Monospecific antibodies, directed against type IV collagen and laminin, were used to clarify the process of sinusoidal capillarization in rats after carbon tetrachloride (CCl4) intoxication by the direct immunoperoxidase method. After acute intoxication, both type IV collagen and laminin were increased in the area of hepatic necrosis, adjacent to the central veins; however, sinusoidal capillarization was not found. During chronic intoxication, deposition of laminin was co-distributed with that of type IV collagen, but deposition proceeded more slowly than that of the type IV collagen. Deposition of laminin was increased in the Disse space. Sinusoidal capillarization was noted as thick deposition of both antigens by light microscopy. Immunoelectron microscopy showed that both components were continuously present in the Disse space. Intracellularly, both antigens were found in the rough endoplasmic reticulum (RER) of fat-storing cells (FSC) and endothelial cells, and these cells showed morphological changes, becoming slender and flattened. In contrast, few immunoreactive products of the two components were observed in the hepatocytes. These findings suggest that type IV collagen and laminin are indispensable for the establishment of sinusoidal capillarization, and that FSC play an important role in the production of both components.</p>","PeriodicalId":18183,"journal":{"name":"Liver","volume":"11 5","pages":"260-71"},"PeriodicalIF":0.0,"publicationDate":"1991-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1111/j.1600-0676.1991.tb00528.x","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13119455","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 22
Hepatic extraction of the aminoterminal propeptide of type III procollagen before and after bile duct ligation in pigs. 猪胆管结扎前后III型前胶原氨基末端前肽的肝脏提取。
Liver Pub Date : 1991-10-01 DOI: 10.1111/j.1600-0676.1991.tb00534.x
K D Bentsen, J H Henriksen, S Boesby, L T Jensen, K Hørslev-Petersen, I Lorenzen
{"title":"Hepatic extraction of the aminoterminal propeptide of type III procollagen before and after bile duct ligation in pigs.","authors":"K D Bentsen,&nbsp;J H Henriksen,&nbsp;S Boesby,&nbsp;L T Jensen,&nbsp;K Hørslev-Petersen,&nbsp;I Lorenzen","doi":"10.1111/j.1600-0676.1991.tb00534.x","DOIUrl":"https://doi.org/10.1111/j.1600-0676.1991.tb00534.x","url":null,"abstract":"<p><p>The aminoterminal propeptide of type III procollagen is extracted from the circulation by the liver, and PIIINP is found in bile. This study was performed in order to investigate whether biliary excretion contributes substantially to the hepatic extraction of circulating PIIINP. Hepatic extraction before and during a 4-h period after ligation of the common bile duct was assessed from serum PIIINP concentrations in a systemic artery, the portal vein and a hepatic vein of seven healthy anaesthetized pigs. Seven sham-operated anaesthetized pigs served as controls. Ligation of the bile duct did not cause a decrease in the hepatic extraction ratio of circulating PIIINP. The PIIINP serum levels of the cholestatic pigs and of the controls were similar throughout the investigation period. The PIIINP concentrations in bile were only 10% of the corresponding serum values. Gel filtration of sera showed that the lower PIIINP concentration in the hepatic vein, as compared to the artery and the portal vein was due to a selective decrease in the concentration of the intact propeptide. The study shows that biliary excretion does not contribute significantly to the hepatic extraction of circulating PIIINP in the normal liver. Furthermore, the hepatic extraction of circulating PIIINP preferentially affects the intact propeptide, rather than the somewhat larger PIIINP related molecule in serum.</p>","PeriodicalId":18183,"journal":{"name":"Liver","volume":"11 5","pages":"310-5"},"PeriodicalIF":0.0,"publicationDate":"1991-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1111/j.1600-0676.1991.tb00534.x","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13119462","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 3
Interferon therapy of chronic delta hepatitis in patients cured of pediatric malignancies: possible harmful effect. 小儿恶性肿瘤治愈后慢性丁型肝炎的干扰素治疗:可能的不良影响。
Liver Pub Date : 1991-10-01 DOI: 10.1111/j.1600-0676.1991.tb00527.x
F Rossetti, F Pontini, C Crivellaro, P Cadrobbi, M Guido, P Pontisso, C Pintus, F Bortolotti, L Zanesco
{"title":"Interferon therapy of chronic delta hepatitis in patients cured of pediatric malignancies: possible harmful effect.","authors":"F Rossetti,&nbsp;F Pontini,&nbsp;C Crivellaro,&nbsp;P Cadrobbi,&nbsp;M Guido,&nbsp;P Pontisso,&nbsp;C Pintus,&nbsp;F Bortolotti,&nbsp;L Zanesco","doi":"10.1111/j.1600-0676.1991.tb00527.x","DOIUrl":"https://doi.org/10.1111/j.1600-0676.1991.tb00527.x","url":null,"abstract":"<p><p>In our Pediatric Haemato-Oncology Unit, 42 young patients cured of their malignancy were left with chronic delta hepatitis. The severity of liver disease in many of these patients prompted us to start a pilot study on the effect of recombinant alpha 2b interferon, given at a dose of 5 MU/square meter thrice weekly. All nine patients included in the study (five males, mean age: 15 years) had well-compensated liver disease, including five cases with active hepatitis and cirrhosis. At the end of the 3rd month of therapy, two patients with cirrhosis developed a biochemical exacerbation leading to hepatic decompensation, which was fatal in one case. The reasons for this unfavourable outcome remain unclear. Basic immunological tests were normal, but one of the two patients was the single case with anti-liver-kidney microsome antibodies. On the other hand, both patients seroconverted from hepatitis B e antigen to antibody at the time of exacerbation, suggesting that liver damage could have been the result of cell-mediated cytotoxicity to hepatitis B virus antigens. The results of this study, which has been interrupted at the 4th month, suggest that interferon therapy for chronic delta hepatitis has to be considered cautiously in young patients cured of pediatric malignancies. In fact, no beneficial effect was seen and the treatment appeared to be harmful in at least two out of nine patients treated.</p>","PeriodicalId":18183,"journal":{"name":"Liver","volume":"11 5","pages":"255-9"},"PeriodicalIF":0.0,"publicationDate":"1991-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1111/j.1600-0676.1991.tb00527.x","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13119516","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 7
Expression of epidermal growth factor and fibroblast growth factor in human hepatocellular carcinoma: an immunohistochemical study. 人肝细胞癌中表皮生长因子和成纤维细胞生长因子表达的免疫组织化学研究。
Liver Pub Date : 1991-10-01 DOI: 10.1111/j.1600-0676.1991.tb00529.x
Y Motoo, N Sawabu, Y Nakanuma
{"title":"Expression of epidermal growth factor and fibroblast growth factor in human hepatocellular carcinoma: an immunohistochemical study.","authors":"Y Motoo,&nbsp;N Sawabu,&nbsp;Y Nakanuma","doi":"10.1111/j.1600-0676.1991.tb00529.x","DOIUrl":"https://doi.org/10.1111/j.1600-0676.1991.tb00529.x","url":null,"abstract":"<p><p>Expression of epidermal growth factor (EGF) and fibroblast growth factor (FGF) was examined in 56 patients with hepatocellular carcinoma (HCC) using an immunohistochemical method. EGF and FGF were expressed on carcinoma cells in 14 (25%) and 23 cases (41%), respectively. In the 23 FGF-positive cases, 11 cases were positive for both acidic and basic FGF, while 18 were positive for acidic FGF, and 16 were positive for basic FGF. In non-cancerous hepatic tissues, FGF was weakly positive in macrophages, hepatocytes and vascular endothelial cells in some cases, while EGF was totally negative. There were no significant correlations between the expression of EGF or FGF on carcinoma cells and the various clinicopathologic factors examined. These data suggest that EGF and FGF are produced by human HCC cells in vivo. The roles of the expression of these growth factors in the development and progression of HCC remain only speculative.</p>","PeriodicalId":18183,"journal":{"name":"Liver","volume":"11 5","pages":"272-7"},"PeriodicalIF":0.0,"publicationDate":"1991-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1111/j.1600-0676.1991.tb00529.x","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"12826070","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 45
Enzyme immunoassay of oestrogen receptors in needle biopsies from human liver. 人肝脏穿刺活检中雌激素受体的酶免疫分析。
Liver Pub Date : 1991-10-01 DOI: 10.1111/j.1600-0676.1991.tb00532.x
U Becker, J Andersen, H S Poulsen, F Burcharth, C Gluud, T Horn
{"title":"Enzyme immunoassay of oestrogen receptors in needle biopsies from human liver.","authors":"U Becker,&nbsp;J Andersen,&nbsp;H S Poulsen,&nbsp;F Burcharth,&nbsp;C Gluud,&nbsp;T Horn","doi":"10.1111/j.1600-0676.1991.tb00532.x","DOIUrl":"https://doi.org/10.1111/j.1600-0676.1991.tb00532.x","url":null,"abstract":"<p><p>For quantitative assessments of sex hormone receptors in liver tissue, ligand binding assays are inconvenient, as they require large biopsies (0.5-1.0 g). The present study shows that it is possible to measure oestrogen receptors (ER) quantitatively in needle biopsy specimens as small as 10 mg by modifications of a commercial enzyme immunoassay employing monoclonal antibodies. Sucrose gradient centrifugation and the dextran charcoal method served as reference methods. A consecutive series of needle biopsies from patients suspected of liver disease were investigated. The biopsies (n = 37) had a median weight of 14 mg and cytosolic protein concentrations greater than 1 mg/ml (median 1.28 mg/ml). The median ER concentration was 20 fmol/mg cytosolic protein (range 5 to 57 fmol/mg). The intra-assay coefficient of variation was 8.9%, the inter-assay 13.2%, and the detection limit 2.7 fmol/ml cytosol. Women had significantly higher ER concentrations (median 22 fmol/mg) compared to male patients (median 16 fmol/mg) (P = 0.007). The enzyme immunoassay measures ER in liver specimens as small as 10 mg, compared to the large tissue specimens necessary for the conventional DCC assay, and the method is a convenient tool for further studies of ER in routine needle biopsies from the liver.</p>","PeriodicalId":18183,"journal":{"name":"Liver","volume":"11 5","pages":"292-9"},"PeriodicalIF":0.0,"publicationDate":"1991-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1111/j.1600-0676.1991.tb00532.x","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13119461","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 3
Histological changes in the liver in experimental graft-versus-host disease across minor histocompatibility barriers. VI. A light and electron microscopic study of the periportal changes. 实验性移植物抗宿主病中肝脏的组织学变化跨越轻微的组织相容性障碍。门静脉周围变化的光镜和电镜研究。
Liver Pub Date : 1991-10-01 DOI: 10.1111/j.1600-0676.1991.tb00530.x
A Nonomura, N Kono, Y Mizukzmi, Y Nakanuma, F Matsubara
{"title":"Histological changes in the liver in experimental graft-versus-host disease across minor histocompatibility barriers. VI. A light and electron microscopic study of the periportal changes.","authors":"A Nonomura,&nbsp;N Kono,&nbsp;Y Mizukzmi,&nbsp;Y Nakanuma,&nbsp;F Matsubara","doi":"10.1111/j.1600-0676.1991.tb00530.x","DOIUrl":"https://doi.org/10.1111/j.1600-0676.1991.tb00530.x","url":null,"abstract":"<p><p>Periportal changes of the liver in experimental graft-versus-host disease (GVHD) across minor histocompatibility barriers were investigated electron-microscopically for up to 14 months after bone marrow transplantation (BMT). In GVHD mice, periportal changes affecting the limiting plate of hepatocytes were relatively mild and, in general, classical piecemeal necrosis was rarely observed. However, around 2 weeks after transplantation disruption of the limiting plate of hepatocytes was transiently observed. At that time, lymphocytes invaded directly into the hepatic parenchyma and were in close contact with hepatocytes mainly through a number of point-contacts of cell membranes. Hepatocytes in close contact with lymphocytes showed minor degenerative changes under electron microscopy. On the other hand, periportal bile ductules and canals of Hering were constantly injured by inflammatory cells during the entire observation period up to 14 months after BMT. They were abutted by lymphocytes, together with other inflammatory cells including eosinophils, neutrophils, plasma cells and monocytes. Infiltration of inflammatory cells into the epithelial layer of the bile ductules and canals of Hering through the basement membrane was frequently found. Inflammatory cells were in contact with duct epithelial cells mainly through a number of point-contacts of cell membranes. Epithelial cells in contact with inflammatory cells exhibited a number of degenerative changes, including condensation of cytoplasm, irregular contour of nucleus, dilatation of endoplasmic reticulum, formation of cytoplasmic vesicles, focal cytoplasmic degeneration, and so on.(ABSTRACT TRUNCATED AT 250 WORDS)</p>","PeriodicalId":18183,"journal":{"name":"Liver","volume":"11 5","pages":"278-86"},"PeriodicalIF":0.0,"publicationDate":"1991-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1111/j.1600-0676.1991.tb00530.x","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13119457","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 6
Changes of cytokeratin filament organization in human and murine Mallory body-containing livers as revealed by a panel of monoclonal antibodies. 单克隆抗体显示人类和小鼠含Mallory体肝脏中细胞角蛋白丝组织的变化。
Liver Pub Date : 1991-10-01 DOI: 10.1111/j.1600-0676.1991.tb00533.x
K H Preisegger, K Zatloukal, G Spurej, H Denk
{"title":"Changes of cytokeratin filament organization in human and murine Mallory body-containing livers as revealed by a panel of monoclonal antibodies.","authors":"K H Preisegger,&nbsp;K Zatloukal,&nbsp;G Spurej,&nbsp;H Denk","doi":"10.1111/j.1600-0676.1991.tb00533.x","DOIUrl":"https://doi.org/10.1111/j.1600-0676.1991.tb00533.x","url":null,"abstract":"<p><p>Mallory bodies (MBs) are characteristics morphologic features of alcoholic hepatitis and can be produced in mouse hepatocytes by chronic griseofulvin (GF) intoxication. The formation of MBs, which share some immunological, biochemical, and ultrastructural features with cytokeratin (CK) filaments of normal liver, is accompanied by derangement and even loss of the CK cytoskeleton of hepatocytes (\"empty cells\") as revealed by immunofluorescence microscopy. To clarify whether this diminution or lack of CK-related staining of MB-containing hepatocytes was due to loss of CK filaments or changes in antigenicity or accessibility of antigenic determinants immunohistochemical studies using a battery of monoclonal and polyclonal CK antibodies were performed. It could be shown that all these antibodies directed against different CK polypeptide components and antigenic determinants of CKs revealed a highly reduced or even undetectable cytoplasmic CK meshwork in most cells with fully developed large MBs. In the light of our present knowledge of the organization of CK intermediate filaments, these results indicate that the phenomenon of the \"empty cells\" reflects a diminution of CK meshwork rather than altered antigenic determinants.</p>","PeriodicalId":18183,"journal":{"name":"Liver","volume":"11 5","pages":"300-9"},"PeriodicalIF":0.0,"publicationDate":"1991-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1111/j.1600-0676.1991.tb00533.x","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"12884097","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 13
Immunological abnormalities in acute post-transfusion hepatitis non-A non-B. 急性输血后非甲非乙肝炎的免疫异常。
Liver Pub Date : 1991-06-01
P Salillas, R Bárcena, M Nocito, J C Erdozain, A Botella, J A Brieva
{"title":"Immunological abnormalities in acute post-transfusion hepatitis non-A non-B.","authors":"P Salillas,&nbsp;R Bárcena,&nbsp;M Nocito,&nbsp;J C Erdozain,&nbsp;A Botella,&nbsp;J A Brieva","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>A variety of immunological parameters has been serially examined in the blood of 12 patients with acute post-transfusion hepatitis non-A non-B (PTHNANB). Several alterations of these tests were transiently detected when comparing with healthy, extensively transfused subjects as well as with normal volunteers: 1. Low proportions of CD8+ T lymphocytes were observed at the disease onset, and these returned to normal after 1 month; 2. A diminution of the proliferative response of blood lymphocytes to mitogens was detected during the same period of time; 3. By the third month of disease, an enhanced spontaneous IgG secretion by cultured lymphocytes was found, and this observation was restricted to those patients who had not recovered. These alterations suggest that the immune system might be involved in the course of hepatitis C virus infection.</p>","PeriodicalId":18183,"journal":{"name":"Liver","volume":"11 3","pages":"170-5"},"PeriodicalIF":0.0,"publicationDate":"1991-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13069378","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Hypoceruloplasminemia and ultrastructural changes resembling Wilson's disease in nonalcoholic liver steatosis. A clinical and pathological study of five cases. 非酒精性肝脂肪变性患者低蓝纤溶酶血症和类似威尔逊氏病的超微结构改变。5例临床病理分析。
Liver Pub Date : 1988-10-01
A P Geubel, V Gregoire, J Rahier, W Lissens, C Dive
{"title":"Hypoceruloplasminemia and ultrastructural changes resembling Wilson's disease in nonalcoholic liver steatosis. A clinical and pathological study of five cases.","authors":"A P Geubel,&nbsp;V Gregoire,&nbsp;J Rahier,&nbsp;W Lissens,&nbsp;C Dive","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>We report five cases of asymptomatic patients with persistently abnormal liver function tests in whom copper metabolism abnormalities resulted in a misleading suspicion of Wilson's disease. Ceruloplasmin levels assessed by nephelometric immunoassay, single radial immunodiffusion and the enzymatic method averaged 53.8, 61 and 52.8% of the mean value obtained in age- and sex-matched controls (p less than 0.001). Twenty-four-hour urinary copper excretion was higher than the normal range in three instances. Four patients exhibited hypertriglyceridemia. Liver histology showed fatty change with or without sinusoidal fibrosis. Electron microscopic examination unexpectedly disclosed mitochondrial and lysosomal changes identical to those described in Wilson's disease. The present observations indicate that biochemical and ultrastructural changes suggestive for Wilson's disease may be observed in the absence of increased liver copper content. Whether such cases represent isolated cases of heterozygosity for the Wilson's disease gene remains to be elucidated.</p>","PeriodicalId":18183,"journal":{"name":"Liver","volume":"8 5","pages":"299-306"},"PeriodicalIF":0.0,"publicationDate":"1988-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"14329194","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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