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Query: UMLS:C0008370 (cholestasis)
 9,378 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

An ultrastructural study of liver cells was performed on 4 patients with primary biliary cirrhosis (PBC) and on 3 patients with chronic active hepatitis (CAH). In liver cells of PBC patients large (ad 2-3 mu in diameter) particles were seen, which morphologically resembed autophagic vacuoles or large secondary lysosymes. The morphology, size, location, and quantity of these particles corresponded to the orcein positive material seen in light microscopy of adjacent sections in specimens from PBC patients. These large particles were not seen in liver cells of CAH patients, which also lacked the orcein positive material in light microscopy. The author suggests that the observed particles indicate the activation of lysosomal compartment in liver cells in PBC, and that the orcein positive material (copper-protein complex accumulating in liver cells in chronic cholestasis) is taken into the phagolysosomal metabolism and processing in liver cells, and possibly resembles in this respect the intracellular metabolism of iron-compounds.
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PMID:Orcein positive hepatocellular material in long-standing biliary diseases. II. Ultrastructural studies. 6 83

Forty-one pregnant women with pruritus, in whom cholestasis was verified by the presence in their serum of an abnormal lipoprotein, lipoprotein-X (LP-X), were divided into two clinical groups, pruritus gravidarum (PG) (n=20) and hepatosis of pregnancy (HP) (n=21) in relation to serum bilirubin (below and above 1.2 mg/100 ml, respectively) and/or SGOT, SGPT (below and above 50 units/l, respectively). In HP, but not in PG, serum lipids, i.e. cholesterol, phospholipids, triglycerides, pre-beta-lipoproteins (very-low-density lipoproteins), and low-density lipoproteins were increased and high-density lipoproteins decreased when compared with suitable controls. Serum lipids were elevated in proportion to the derangement in the liver function tests, alkaline phosphatase, SGOT, and SGPT. The occurrence of LP-X was inversely related to HDL cholesterol, suggesting a causal relationship between HDL lipid metabolism and the presence of LP-X. Serum TIBC, Simplastin A, and serum iron were elevated in HP in relation to the degree of deterioration of liver function tests. Some of these changes in serum in cholestatic pregnancy may partially (serum triglycerides and pre-beta-lipoproteins) or completely (TIBC and Simplastin A) be explained by an enhanced estrogen influence in promoting increased liver lipid/protein metabolism.
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PMID:Studies in cholestasis of pregnancy. 16 48

Three cases of chronic nonhaemolytic jaundice with conjugated bilirubin in the serum are described in a Chinese family. Bromsulphthalein excretion tests gave results typical of the Dubin-Johnson syndrome. Liver histology in the proband showed cytoplasmic pigment of the lipofuscinmelanin variety, and intravenous cholecystography failed to show visualisation of the gallbladder. Unusual findings included onset during the neonatal period in the proband and the presence of some iron pigment in the hepatic cells with a little canalicular cholestasis. It is suggested that the infant may have had a concomitant nonspecific hepatitis. These cases are regarded as belonging to a disease group in which the Dubin-Johnson syndrome is at one end of a spectrum. The mode of inheritance is discussed.
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PMID:Dublin-Johnson syndrome with some unusual features in a Chinese family. 48 95

A rational diagnostic procedure has to be not only scientifically should but also economically reasonable. One has always to ask to what purpose the diagnosis shall serve. The diagnosis is mainly necessary for the treatment of a patient. This includes aiming at a causal therapy, informing the patient about cause and meaning of his symptoms, and also considering prognosis and, if necessary, prophylaxis. In liver disease the following investigations are obligatory: history, signs and symptoms and a minimal set of tests (SGPT, SGOT, gamma-GT, serum bilirubin, urobilinogen in the urine). The next level of diagnostic measures evolves out of several questions: in case of acute disease: etiology (infections; toxic?); evidence of chronic liver disease; differentiation between intra- and extrahepatic cholestasis; evidence of a circumscript lesion of the liver; associated reaction of the liver in connection with other extrahepatic diseases. Serum bilirubin is of relatively little importance except for disturbances of the bilirubin metabolism. The same is true for serum iron except for the diagnosis of hemochromatosis. Blood coagulation tests are of great value for the diagnosis and evaluation of acute and chronic liver disease as are immunologic and serologic investigations (HBsAg, HBcAg, Anti-HBeAg, Anti-HVA, ANF, SMA).
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PMID:[Rational diagnosis in liver diseases]. 64 May 55

The non-invasive diagnostic technique of computed tomograph (CT) has been assessed in 100 patients with established liver disease. CT can differentiate extra- from intrahepatic cholestasis and may define the obstructing lesion in the former group. It is of value in detecting infiltrations with fat or iron, and provides useful information in patients with cirrhosis and metastatic deposits. At present it appears of less value in the diagnosis of non-fibrotic parenchymal liver disease.
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PMID:Computed tomographic scanning in liver disease. 64 84

Histologic liver examinations performed in 500 autopsies showed 32 times the incidence of cholestatic hepatosis. Males are affected more commonly than females as our statistic indicates. Furthermore it was shown that chronic alcoholism seems to be a more aggravating cause for hepatosis than a long term treatment with psychopharmacologic drugs. No correlation however was found between the extent of cholestasis and morphologic alterations (inflammation, fatty degeneration, iron storage and necrosis).
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PMID:[The histo-morphologic patterns of cholestatic hepatosis (author's transl)]. 88 52

Two premature female infants with a new variant of pulmonary hyaline membrane disease had both received assisted ventilation with high oxygen concentrations. Postmortem examination showed straw-yellow hyaline membranes lining the terminal bronchioles and alveoli. Pneumonocytes and macrophages also contained straw-yellow pigments in the cytoplasm. These yellow membranes and pigments in pneumonocytes and in macrophages stained positively for bile and negatively for iron. Both infants had severe intrahepatic cholestasis. Cholestasis and pulmonary changes due to oxygen toxicity are believed to be the main features leading to this curious entity.
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PMID:Pulmonary yellow hyaline membrane disease. New variant in premature infants with intrahepatic cholestasis. 94 1

Pregnant women with pruritus and with cholestasis of pregnancy verified from the medical history and by the presence of lipoprotein-X in serum have been studied. Thirty-nine patients were investigated for serum lipids (cholesterol, phospholipids, and triglycerides), serum lipoproteins (high-density-lipoproteins cholesterol and estimated low-density-lipoproteins cholesterol), and for hematological data (serum iron and serum iron binding capacity), in relation to duration of pruritus. In 28 patients the serum lecithin concentrations and fatty acid compositions were also analysed. Severity and duration of the disease appeared to influence the lipid/lipoprotein metabolism. The results support the hypothesis of an abnormal reaction of liver metabolism to estrogens in the initial stage of cholestasis of pregnancy.
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PMID:Studies in cholestasis of pregnancy. IV. Serum lipids and lipoproteins in relation to duration of symptoms and severity of the disease, and fatty acid composition of lecithin in relation to duration of symptoms. 119 99

Mitochondrial cytopathies are multisystemic diseases of extremely variable expression caused by a deficiency in oxidative phosphorylation. Only five cases of neonatal liver failure in the context of mitochondrial cytopathy have been reported, with incomplete morphological data of the liver in three. In the case presented here, ascites had been diagnosed prenatally and liver failure was particularly severe (factor V less than 15% with fatal coma the fourth day). Histologically there were incomplete cirrhosis, microvesicular steatosis, major canalicular cholestasis with proliferative neocholangioles, and bile duct thrombi. There were also some iron pigments in the periportal area and partial glycogen depletion. By electron microscopy, mitochondria in numerous hepatocytes appeared abnormal with occasional cristae in a fluffy matrix, some containing dense inclusions. Study of respiratory chain activity showed a defect in cytochrome c oxidase (complex IV), revealed by oxygraphic measurement on fresh muscle biopsy and confirmed by spectrophotometric enzymatic assays performed on muscle and liver homogenates. The association of neonatal liver failure with hyperlactacidemia warrants investigation into a deficiency in oxidative phosphorylation.
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PMID:Fatal neonatal liver failure and mitochondrial cytopathy: an observation with antenatal ascites. 139 93

To evaluate malnutrition in chronic liver disease, and its relationship to nutrient deficiencies and hepatic dysfunction, 27 children with end-stage liver disease were studied. Mean protein-energy intakes were 70% of recommended daily intakes. The patients were underweight and stunted with reduced mean triceps and subscapular skinfold thicknesses and midupper arm circumference. Mean total body potassium was only 63 +/- 18% of that expected for age and sex. Deficiency of essential fatty acids (32%), and low concentrations of fat-soluble vitamins (A, 92%; E, 32%), iron (32%), zinc (42%), and selenium (13%) were common. Serum ammonia concentrations were raised in all patients, and increased methionine, tyrosine, and glutamic acid, and reduced glutamine concentrations were noted. There was no correlation between the degree of malnutrition and the degree of liver synthetic function, the degree of cholestasis, or the degree of liver injury. We suggest that potentially correctable factors in addition to liver failure (eg, inadequate absorbed intake) were important determinants of malnutrition in these patients.
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PMID:The nature of malnutrition in children with end-stage liver disease awaiting orthotopic liver transplantation. 160 54


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