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)

The object of the investigation was to study the capacity of the liver to metabolize drugs under diversified conditions (cirrhosis, hepatitis, cholestasis in alcoholics, in diabetics as well as in eplieptics treated with barbiturates) as well as the possibility of inducing this function. To accomplish this we determined the half-life of Butazolidin. Only in cirrhotics it was shown that the capacity to metabolize Butazolidin was reduced. Induction with barbiturates increased hepatic captation of the drug but did not modify the biotransformation of the same. This is attributed to the development of a "hypoactive hypertrophy" of the reticuloendothelium.
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PMID:[Metabolism of phenylbutazone in the liver (author's transl)]. 95 7

The apparent well-being of some children who as neonates were believed to have obstructive jaundice prompted us to study the clinical course, histologic features and possible etiologic factors in 17 children with cholestasis in the neonatal period. During a follow-up period of five months to 22 years, all had signs of chronic cholestasis, but only four died (two from nonhepatic causes); the others live remarkably normal lives. Serial hepatic biopsies in 11 showed a variety of initial lesions, which progressed to hypoplasia of the intrahepatic bile ducts, increasing portal fibrosis and eventual cirrhosis. Although evidence of possible viral infection was found in only 10 cases, a hepatitis, beginning either before or after birth, appears to be a likely original cause. The histologic changes seen may represent different stages of one process, starting as cholestasis with or without evidence of hepatitis and progressing to obliteration or failure of normal growth of the intrahepatic bile ducts.
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PMID:Intrahepatic cholestasis in childhood. 95 76

Samples of serum, bile, and urine were collected simultaneously from patients with cholestasis of varying aetiology and from patients with cirrhosis; their bile acid composition was determined by gas/liquid chromatography and mass spectrometry. In cholestasis, the patterns in all three body fluids differed consistently and strikingly. In serum, cholic acid was the major bile acid and most bile acids (greater than 93%) were unsulphated, whereas, in urine, chenodeoxycholic was the major bile acid, and the majority of bile acids (greater than 60%) were sulphated. Secondary bile acids were virtually absent in bile, serum, and urine. The total amount of bile acids excreted for 24 hours correlated highly with the concentration of serum bile acids; in patients with complete obstruction, urinary excretion averaged 71-6 mg/24 h. In cirrhotic patients, serum bile acids were less raised, and chenodeoxycholic acid was the predominant acid. In healthy controls, serum bile acids were consistently richer in chenodeoxycholic acid than biliary bile acids, and no bile acids were present in urine. No unusual monohydroxy bile acids were present in patients with primary biliary cirrhosis, but, in several patients, there was a considerable amount of hyocholic acid present in the urinary bile acids. The analyses of individual bile acids in serum and urine did not appear to provide helpful information in the differential diagnosis of cholestasis. Thus, in cholestasis, conjugation of chenodeoxycholic acid with sulphate becomes a major biochemical pathway, urine becomes a major route of bile acid excretion, and abnormal bile acids are formed.
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PMID:Sulphated and unsulphated bile acids in serum, bile, and urine of patients with cholestasis. 100 76

A child, two months of age, suffering from cholestasis and biliary cirrhosis, was found to be homozygous for alpha1-antitrypsin deficiency Pi type ZZ associated with high degree extrahepatic bile duct obstruction. The clinical, protein-chemical, genetic, histological and immuno-histochemical findings in the patient are reported and the relationships between these two anomalies interpreted. An interaction between the very rare defects rather than random association is suggested.
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PMID:Hereditary alpha1--antitrypsin deficiency associated with congenital extrahepatic bile duct hypoplasia. 107 95

The authors report a case of neonatal hepatitis with alpha-1-antitrypsin occuring in a child of ZZ phenotype. The anatomopathological study carried out on two liver biopsies showed changes of common cholestatic hepatitis developing into cirrhosis, as well as intrahepatocytary globulins. Moreover, these globulins, P.A.S. positive after treatment by alphaamylase, fix an antialpha-1-antitrypsine antiserum. Ultrastructural analysis shows them to be masses of amorphous material, feebly osmiophilic, outlined by a unitary membrane the moniliform aspect of which recalls the ergastoplasmic membrane. These findings are identical to those already made in cases of cirrhogenous neonatal hepatitis by alpha-1-antitrypsine deficit reported in the literature. They point out the irreversibility of the affection which, after a stage of cholestatic hepatitis with or without inflammatory portal fibrosis, develops into cirrhosis. At this stage cholestasis has regressed or disappeared whereas portal sclerosis, often infiltrated with free elements, surrounds hepatic lobules and biliary neocanaliculi. But the globulins are still present and appear to be the specific feature of this deficit. By their ultrastructural and immuno-histochemical features, these globulins would represent a form of accumulation of alpha-1-antitrypsin in the hepatocytes which normally carry out the synthesis of this antienzyme. Accumulation in the hepatocytes proves excretory disturbance of hypothetical mechanism: structural anomaly, changes in the permeability of the membrane. Its role in the occurrence of hepatitis or cirrhosis lesions is still to be demonstrated but one may think that it consists in absence of inhibition of the enzymatic factors discharged during agressions.
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PMID:[Neonatal hepatitis with alpha-1-antitrypsin deficit. Apropos of a personal case]. 108 58

Problems of pregnancy in patients with liver disease are discussed. The effects of pregnancy on the disease course are generally limited to triggering of intensifiying icterus and pruritus; intrahepatic cholestasis may also occur. Increased incidences of miscarriage and prematurity have been reported in patients with liver cirrhosis, chronic hepatitis, cholestasis, and Dubin-Johnson syndrome, which is hereditary, and viral hepatitis in early pregnancy (increased incidence of chromosome abnormalities). Liver diseases constitute a relative indication for abortion, depending on the general state of the mother's health and her desire for the child. Problems of diagnosis and treatment are also considered.
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PMID:[Pregnancy in liver diseases]. 112 34

Focal biliary cirrhosis is an uncommon finding in infants with cystic fibrosis, but it is present in more than a fifth of surviving children and adolescents. It was found at postmortem examination in only five of 47 infants with CF younger than 3 months, in five of 32 infants from 3 to 12 months, and in 18 of 67 children older than 1 year. In infants under 3 months, excessive mucus in intrahepatic bile ducts was seen in 11 necropsies; in 15 others there were only nonspecific periportal changes. Cholestasis was found in the livers of 18 of the 26 infants. Excessive mucus in the biliary tree was occasionally associated with periportal changes and cholestasis in older infants. The periportal changes, which are regarded as nonspecific, were never found in infants more than 1 year of age.
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PMID:Hepatic changes in young infants with cystic fibrosis: possible relation to focal biliary cirrhosis. 113 49

In 36 children with cystic fibrosis (CF) the isoenzymes of alkaline phosphatase (AP) were determined microelectrophoretically in polyacrylamide- and starch-gel. The study was done to evaluate the clinical significance of these additional data for the diagnosis of liver involvement in DF. The results led to the following conclusions: 1. Serum activity of total AP is comparatively unsensitive "masking" alterations in the isoenzyme pattern contributing to the AP serum activity. 2. In 17 children resp. 47% bile-duct phosphatase was increased indicating a secretostasis while other marker enzymes of cholestasis were normal in part. 3. The activity of bone phosphatase in the serum showed a significant correlation to the degree of growth retardation in these patients. 4. Intestinal phosphatase was present in the serum of only one child with cirrhosis of the liver being an indicator for liver insufficiency. 5. Determination of AP isoenzymes in the serum may provide additional information about the organs involved for the physician in handling CF patients.
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PMID:Isoenzymes of alkaline phosphatase in the serum of patients with cystic fibrosis. 113 40

The authors present an experimental series of prosthetic replacement of the common bile duct in the dog. The material used was silicone elastomere. In all the dogs studied, a new bile duct became formed around the prosthesis consisting of a fibrous tube covered with mucosa. The follow-up period in the first group of operated dogs was more than 2 1/2 years and in most dogs studied, there was neither biliary cirrhosis nor cholestasis. These encouraging results led the authors to suggest the operation in humans, particularly in carcinoma of the bile ducts.
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PMID:[Segmental replacement of the prinicipal extra-hepatic bile duct in the dog using a silicone elastomere prosthesis]. 115 Jul 60

Outbred albino mice were rendered protoporphyric by a diet containing 2.5% (weight) of griseofulvin. There was a 5-fold increase in liver weight, hepatocellular degeneration and necrosis, cholestasis, ductular proliferation and cirrhosis. Liver protoporphyrin values were elevated and brown pigment granules were present in hepatocytes, Kupffer cells, and bile ducts. The granules showed red fluorescence, birefringence, and, at the ultrastructural level, consisted of aggregates of needle-like crystals. Crystals isolated from such livers showed solubility and absorption characteristics of protoporphyrin; in vitro recrystallization of protoporphyrin, extracted from protoporphyric mouse livers, yielded crystals identical with those observed in vivo, and commercial protoporphyrin exhibited similar morphologic features. The liver pathology and protoporphyrin crystals observed in these animals are identical to the liver pathology and crystals observed in the human disease, erythropoietic protoporphyria. In this mouse model, protoporphyrin crystals are intimately associated with hepatocellular injury and it appears that their accumulation within hepatocytes leads to hepatocellular destruction. A similar pathogenesis is postulated for the hepatic damage that occurs in some cases of erythropoietic protoporphyria.
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PMID:Mouse model for protoporphyria. I. The liver and hepatic protoporphyrin crystals. 115 16


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