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)

An ultrastructural morphometric study was carried out to detect differences in parenchymal structures between chronic viral hepatitis and hepatitic cirrhosis for more reliable diagnosis of cirrhosis in needle biopsy. Five human liver biopsy specimens were selected from each of the following five groups: chronic persistent hepatitis (CPH), mild chronic active hepatitis (MCAH), severe chronic active hepatitis (SCAH), active hepatitic cirrhosis (AHC) and inactive hepatitic cirrhosis (IHC). The ultrastructural changes in hepatitic cirrhosis which differed from those in chronic hepatitis were: (1) increase in the volume fraction (Vv) of hepatic intercellular space with increased Vv of collagen fibers and less microvilli in the space; (2) decrease in the Vv of perisinusoidal space with more collagen fibers and less microvilli; (3) decreased Vv and surface density (Sv) of RER in hepatocytes; (4) decreased Vv without change in Sv of mitochondria of hepatocytes.
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PMID:[Ultrastructural morphometry of liver parenchyma in hepatitic cirrhosis]. 208 47

The diagnosis of liver cirrhosis depends on assessing fibrosis and architectural alterations of the liver. In a needle biopsy specimen the connective tissue is often inadequately sampled, which leads to an uncertain diagnosis. Parenchymal features alone are currently insufficient. We, therefore, carried out a comprehensive morphometric study to detect parenchymal structures that may be useful in the diagnosis and prognosis of cirrhosis. Five human liver biopsy specimens were selected from each of four alcoholic disease groups: fatty liver, alcoholic hepatitis, cirrhosis with greater-than-5-yr survival, and cirrhosis with less-than-2-yr survival. Volume fractions (Vv) and surface densities (Sv) were determined stereologically for parenchymal and hepatocellular compartments in electron micrographs. The differences between noncirrhosis and cirrhosis were (a) a doubling of the Vv of parenchymal interstitial space, (b) a nearly 25% increase in the Sv of hepatocyte plasma membrane, (c) a nearly 50% increase in the Sv of hepatocyte RER, and (d) a decrease in the Sv of the outer mitochondrial membrane. The significant difference between the greater than 5-yr and the less than 2-yr survivors of cirrhosis was the marked decrease in hepatocyte nuclear Vv in the latter group. Statistical analysis of our data showed that optimal sampling is achieved with as few as three micrographs from one block of tissue per biopsy specimen.
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PMID:Morphometry of liver parenchyma in needle biopsy specimens from patients with alcoholic liver disease: preliminary variables for the diagnosis and prognosis of cirrhosis. 266 43

Storage of alpha-1-antitrypsin (AAT) has been found in a small number of bile duct cells in liver tissue specimens from patients with Pi MZ, Pi SZ and Pi ZZ phenotypes. The storage appeared in the form of intracellular AAT immunoreactive inclusions. On EM investigation, AAT-like material was detected within cisternae of the RER and SER. Such AAT inclusions were found in proliferating bile ductules in conditions such as cirrhosis, focal nodular hyperplasia and extrahepatic obstruction. They were also observed in normal biliary structures at the level of the canals of Hering, bile ductules and interlobular ducts in 13 out of 47 cases. These findings are interpreted as indicating that the intrahepatic bile duct cells are a further source of AAT, and that in case of defective export of AAT from the cell, as is the case for the Z protein, the protein accumulates not only in hepatocytes but in biliary cells as well.
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PMID:Storage of alpha-1-antitrypsin in intrahepatic bile duct cells in alpha-1-antitrypsin deficiency (Pi Z phenotype). 298 2

The purpose of this discussion is to document the finding of contractile fibroblasts and to examine their possible origin in a further pathological process: focal nodular hyperplasia (FNH) of the liver. A 31-year old female patient underwent laparotomy for a hypochondrial mass. The patient had been taking OCs for 1 year. The mass was located in the right lobe of the liver and excision of the tumor was performed. Some distance from it a subcapsular hemangioma was discovered and resected. After 16 months of follow-up the patient is well. The tumor mass (10 cm diameter) grey in color, was very well demarcated, although not encapsulated. A central scar was present. Radiating septa divided the periphery of the mass into multiple, variable sized modules, simulating a pattern of focal cirrhosis. No areas of hemmorhage or infraction were observed. The hemangioma specimens showed multiple cyst-like cavities filled with blood. The bosselated and lobulated appearance was because of areas of fibrosis, which was most marked centrally. Vascular changes similar to those described by Mays et al. and bile ductular proliferation analogous to that described by others were seen. Single small bile ductules were frequently observed within parenchymal nodules, without any relation to the intranodular scars. Cells with elongated, cross banded nuclei were in close vicinity of the newly formed ductules. Inflammatory and fibroblast-like cells were seen in the fibrous connective tissue septa. The 2nd lesion showed the typical features of a cavernous hemangioma. Typical fibroblasts were identified by their elongated or star-like shape, the slender fusiform shape and relatively smooth outline of the nucleus, the well developed RER and Golgi apparatus, the scattered mitochondria, and the absence or scanty presence of cytofilaments. A large number of elongated cells exhibited the characteristic morphology of modified contractile fibroblasts (MF). They showed a variable amount of intracytoplasmic microfilaments, arranged in longitudinal bundles, usually parallel to the long axis of the cell predominantly located beneath the plasma membrane. Intracellular connections between modified fibroblasts were frequently observed; they were mostly of the macula-adherens type. The finding of contractile fibroblasts raises questions concerning the origin and the significance of such cells in FNH. Since MFs are similar to both fibroblasts and smooth muscle cells, it seemed reasonable to conclude that both cells, under appropriate conditions, may become MF. Fat storing cells of Ito may also be a progenitor of contractile fibroblasts.
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PMID:Myofibroblasts in focal nodular hyperplasia of the liver. 688 82

Alpha-1 antitrypsin deficiency is a frequent genetic disorder associated with pulmonary emphysema in smokers and with liver cirrhosis. Aside from lung or liver transplantation, only replacement therapy can currently slow the progression of emphysema. Progress in the pathogenesis of this disorder (protein misfolding, RER aggregation) is opening the way to new strategies such as proteostasis control. Alpha-1 antitrypsin deficiency remains poorly known and underdiagnosed.
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PMID:[Alpha-1 antitrypsin deficiency 50 years later]. 2675 7