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  • 1
    ISSN: 1600-065X
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary: Primary biliary cirrhosis (PBC) is an organ-specific autoimmune disease that predominantly affects women and is characterized by chronic progressive destruction of small intrahepatic bile ducts with portal inflammation and ultimately fibrosis. The serologic hallmark of PBC is the presence of antibodies to mitochondria, especially to the E2 component of the pyruvate dehydrogenase complex. The mechanisms by which (and if) such antibodies produce liver tissue injury are unknown. However, the presence of these antibodies has allowed detailed immunological definition of the antigenic epitopes, the nature of reactive autoantibodies and the characterization of T-cell responses. Several mechanisms may now be proposed regarding the immune-mediated bile duct damage in PBC, including the possible role of T-cell-mediated cytotoxicity and intracellular interaction between the IgA class of antimitochondrial antibodies and mitochondrial autoantigens. There are major questions which remain unanswered, including, of course, etiology, but also the reasons for female predominance, the absence of PBC in children, the relative ineffectiveness of immunosuppressive drugs, and the specific role of mitochondrial antigens. The data so far provide suggestive evidence that PBC is a mucosal disease; this thesis provides a basis for discussion of etiology via the enterohepatic circulation of toxins and/or infection.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Journal of hepato-biliary-pancreatic surgery 7 (2000), S. 542-550 
    ISSN: 1436-0691
    Keywords: Key words Biliary epithelial dysplasia ; Chronic cholangitis ; Intrahepatic bile duct ; Intrahepatic cholangiocarcinoma ; Precancerous lesion
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Intrahepatic cholangiocarcinoma (ICC), also known as cholangiocellular carcinoma or peripheral bile duct carcinoma, is an intrahepatic malignant tumor that consists of cells resembling the biliary epithelium. The proportion of ICC among primary hepatic malignancies is approximately 10% worldwide. Although the etiology and pathogenesis remain unclear in a great majority of cases, preceding pathologic conditions or etiologies in the development of ICC are known or suspected in a proportion of these patients. Some forms of ICC, such as those associated with hepatolithiasis or liver fluke infestation, are endemic in parts of the world, particularly in East Asia. ICC is reportedly a late complication of primary sclerosing cholangitis. Most of these preceding pathologic conditions are forms of chronic cholangitis, and longstanding inflammation, chronic injury, and regenerative hyperplasia of the biliary epithelium may be causally related to malignant transformation. Biliary epithelial dysplasia is encountered in the intrahepatic bile ducts both near and remote from ICC foci in the liver and near and remote from the chronically inflamed biliary tree. This lesion could be a precancerous lesion, and it shows telomerase activity and increased proliferative activities. Furthermore, congenital and developmental disorders of the biliary system, such as Caroli's disease, congenital hepatic fibrosis, and pancreatico-biliary malformation are occasionally associated with ICC. Benign biliary tumors, such as biliary papillomatosis, may eventually undergo malignant transformation. These lesions could be regarded as precancerous or borderline lesions. Some ICCs may also develop in nonbiliary viral cirrhosis. In the majority of cases of ICC, however, the etiology, pathogenesis, and developmental processes and precancerous lesions of the ICC remain unclear.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-2307
    Keywords: Nodular regenerative hyperplasia of the liver ; Liver metastasis ; Pancreatic endocrine tumour
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Two autopsy cases with multiple hepatic metastases of pancreatic endocrine tumours and nodular regenerative hyperplasia of the liver (NRH) are reported. The tumour cells were positive for glucagon, insulin, gastrin and vasoactive intestinal polypeptide immunohistochemically and the serum gastrin was elevated in one case. In the other, tumour cells were positive for insulin. Controls failed to show NRH in the non-metastatic part of 35 autopsies of livers with multiple hepatic metastases. A combination of hepatotrophic hormonal factor(s) and disturbed hepatic circulation associated with hepatic metastases may be important in the development of NRH.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Virchows Archiv 382 (1979), S. 21-30 
    ISSN: 1432-2307
    Keywords: Chronic intrahepatic cholestasis ; Primary biliary cirrhosis ; Hepatocellular orcein positive granules ; Copper hepatocytotoxicity
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The morphological characteristics of orcein positive granules in hepatocytes from 11 patients with chronic intrahepatic cholestasis and from 2 newborn normal livers were studied. Histochemical investigations revealed their protein nature and many sulphydryl and/or disulphide groups. Copper was demonstrated in the granules by histochemical techniques and electron X-ray micronalysis. No difference was observed in the hepatic distribution and appearance of the granules between the livers of those with chronic cholestasis and the newborn. Ultrastructurally, a variety of electron dense granules were seen at the site of orcein positive granules in the hepatocytes of the patient with primary biliary cirrhosis. Some had a single-layered membrane and seem to be lysosomal derivatives. It is suggested that the copper in lysosomes seen in both chronic cholestasis and normal newborn livers, need not to be cytotoxic.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-2307
    Keywords: Primary biliary cirrhosis ; Ultrastructure of bile duct ; Bile flow disturbance ; Intrahepatic biliary tree
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Using wedge liver biopsies from patients with primary biliary cirrhosis (PBC), ultrastructural features of the intrahepatic bile ducts in livers with slight or no bile duct loss were compared with those in livers with advanced bile duct loss and in extrahepatic cholestasis (EHC). Most changes in the biliary epithelium in PBC were similar to those in EHC. Microvillous loss and bleb formation, mitochondrial damage and increase in endoplasmic reticulum and ribosomes were found in PBC irrespective of the degree of bile duct loss, and also in EHC. These changes were present almost equally at any level of the biliary tree, and are presumed to represent a variety of non-specific lesions of biliary epithelial cells. As the loss of bile ducts in PBC progressed, cytoskeletal filaments and cytophagosomes increased in number and basement membranes were more thickened and reduplicated. These changes were more or less conspicuous in smaller branches of the biliary tree, and were also prominent in EHC. They might be causally related to the bile flow disturbance in the liver. Lateral intercellular spaces were irregularly dilated and contained osmiophilic membranous and/or granular material, similar to that found in duct lumena, within and without the basement membrane, and in the cytoplasm of periductal macrophages. Furthermore, pinocytotic vesicles were increased in the biliary cytoplasm facing periphery. These findings suggest possible alteration of the permeability of biliary epithelial cells, probably in the direction from the lumena to the periductal tissue. Such changes were found in PBC livers with virtual absence of bile duct loss, and the significance of this phenomenon is discussed.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    Virchows Archiv 409 (1986), S. 365-373 
    ISSN: 1432-2307
    Keywords: Hepatocellular carcinoma ; Cholangiocarcinoma ; Human chorionic gonadotropin ; Immunohistochemistry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Production of human chorionic gonadotropin (hCG) by extra-gonadal tumours is not a rare phenomenon. In the liver, similar results have been reported in hepatoblastomas. The present study was attempted to survey hCG level in serum and hCG-immunoreactivity in primary liver carcinoma in adults. Although hCG was elevated in serum in 2 (22.2%) of 9 autopsied cases with hepatocellular carcinoma (HCC), the hCG-reactivity of carcinoma cells was found in 2 (2.1%) of 95 HCC cases. Carcinoma cells positive for immunoreactive hCG was found in 2 (15.4%) of 13 cases with cholangiocarcinoma (CC). The patients with hCG-immunoreactivity in carcinoma and/or elevated serum level of hCG failed to reveal distinct clinical and endocrinological disturbance due to excess hCG. The hCG-positive cells were focal within the carcinoma and showed poor histological differentiation in both HCC and CC, and there were no trophoblastic cells. It is suggested that hCG is one of the hormones produced by primary liver carcinoma in adults and can be localised immunohistochemically in a small number of poorly differentiated carcinoma cells.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Virchows Archiv 414 (1988), S. 53-59 
    ISSN: 1432-2307
    Keywords: Hepatolithiasis ; Intrahepatic biliary tree ; Bacterial infection
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Hepatolithiasis is a common disease in East Asia though very rare in the West. Four cases of hepatolithiasis in which calculi were incidentally found in the peripheral branches of the intrahepatic biliary tree at autopsy are described and compared with hepatolithiasis involving the major branches of the intrahepatic biliary tree. These four cases were all elderly, three patients were male and one female. The calculi were brown pigment stones in each case, as seen in the major branch type. The stone-containing ducts showed mild fibrosis and glandular proliferation with inflammatory changes in three cases; these changes were marked in the fourth case. The hepatic parenchyma around the stone-containing ducts was atrophic or collapsed in all four cases. The major branches of the intrahepatic biliary tree as well as the extrahepatic tree failed to show findings suggestive of bacterial infections or biliary anomalies. These data suggest that brown pigment stones develop primarily in the peripheral ducts in the liver. It remains uncertain whether the peripheral type eventually progresses to the major type or not.
    Type of Medium: Electronic Resource
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  • 8
    ISSN: 1432-2307
    Keywords: Fatty change of the liver ; Macroregenerative nodule of the liver ; Borderline lesion ; Hepatocellular carcinoma
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We here describe the morphologies of 9 macroregenerative nodules (MRNs) showing moderate to marked fatty change (fatty MRN) from 6 cases of non- or minimally-steatotic cirrhotic livers. In most of these cases, no obvious steatogenic factors of the liver were obtainable. These fatty MRNs showed more or less a sharp border. Seven of these fatty MRNs showed a variable degree of unusual morphological alterations suggestive of neoplasia: atypical and hyperchromatic nuclei, abnormal blood vessels, foci of clustering Mallory bodies, numerous hyaline globules, α-fetoprotein-positive hepatocytes, resistance to iron accumulation, infiltration into the portal tracts within MRN, and occurrence of hepatocellular carcinoma without fatty change. These observations suggest that at least some of the fatty MRNs are neoplastic or belong to a borderline lesion, and that the fatty change in the MRNs may be one of hepatocellular expressions related to human hepatocarcinogenesis.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    Virchows Archiv 418 (1991), S. 401-404 
    ISSN: 1432-2307
    Keywords: Adenomatous hyperplasia ; Hepatocellular carcinoma ; DNA ; Cytophotometry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The possibility of adenomatous hyperplasia (AH) being a precusor lesion of hepatocellular carcinoma (HCC) in human cirrhotic livers was investigated. Feulgen DNA cytophotometry was used to measure the DNA content of the hepatocytes in 13 AH nodules obtained from six cirrhotic livers. DNA distribution patterns were classified into types I (diploid pattern), II (hyperploid pattern) and III (aneuploid pattern). According to the cellular and structural atypia, AH nodules were divided into ordinary type (2 nodules) and atypical type (11 nodules), 6 of the latter possessing foci of apparent HCC within them. Two ordinary AH nodules showed a type I DNA distribution pattern, similar to the surrounding regenerative nodules. A major part of the atypical AH nodules also showed type I. However, small foci showing moderate and structural atypia within these atypical AH nodules presented a type I pattern with more hyperploid cells and some aneuploid cells and also a type II histogram pattern with some aneuploid cells. Neoplastic foci, found within 5 atypical AH nodules, displayed various patterns (type I, II, III) as seen in well-developed HCC nodules. These data may imply that atypical AH nodules are precursor lesions of HCC, or are actually undergoing malignant transformation. It is apparent that at least some HCCs occurring in liver cirrhosis evolve through AH.
    Type of Medium: Electronic Resource
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  • 10
    ISSN: 1432-2307
    Keywords: Hepatic cysts ; Mucin histochemistry ; Immunohistochemistry ; Intrahepatic bile ducts
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Epithelial cells of several types of hepatic cysts were examined by mucin histochemistry and immunohistochemically. There were some differences in mucus and antigenic expression among the hepatic cysts examined. Epithelial cells of non-parasitic simple cysts and adult-type polycystic liver showed similar mucin-histochemical and immunohistochemical features, and were characterized by little mucin and weak immunoreactivities to several antibodies examined. Epithelial cells of hepatic hilar cysts were characterized by much mucin and moderate immunoreactivities to carbohydrate antigen 19-9 (CA 19-9), carcinoembryonic antigen (CEA) and epithelial membrane antigen (EMA). Epithelial cells of ciliated hepatic foregut cysts were characterized by much mucin and immunoreactivities to actin and tubulin which were positive in cilia. Epithelial cells of biliary cystadenoma were characterized by much mucin and moderate to strong immunoreactivities to cytokeratins CAM5.2 and AE1 and 3 as well as to CA 19-9, CEA, EMA and DU-PAN-2. Epithelial cells of biliary cystadenocarcinoma were characterized by much mucin and moderate to strong immunoreactivities to cytokeratins CAM5.2 and AE1 and 3 as well as to CA 19-9, CEA, EMA and DU-PAN-2. These differences in epithelial mucus and antigenic expression among several types of hepatic cysts may reflect differences in their origin and biological characteristics. These differences may be helpful in the differential diagnosis of hepatic cysts in small biopsy specimens.
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