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)

Liver biopsies obtained during the first six months of life were studied in 15 children who had prolonged neonatal cholestasis and alpha-1-antitrypsin deficiency (Pi ZZ). Intracellular PAS-positive globules were always observed, even during the first months of life. At this early stage of the disease, three morphologic patterns of hepatic alteration were distinguished. Group 1: six cases with cholestasis, hepatocellular damage, and slight portal fibrosis; Group 2: five cases with important portal fibrosis and bile duct proliferation; and Group 3: four cases in which ductular hypoplasia was the main feature. A correlation between histologic patterns and clinical course may be established: improvement of liver injury in Group 1, early cirrhosis in Group 2, and prolonged cholestasis in Group 3. The natural evolution of the disease appears to be different in each of the three groups.
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PMID:Histopathologic study of the liver in the early cholestatic phase of alpha-1-antitrypsin deficiency. 18 52

Significant liver disease developed in 14 patients after renal transplantation. Nine patients had morphologic and functional evidence of chronic active hepatitis. In general, these patients had few symptoms of liver disease, even though the course of chronic active hepatitis was progressive. Despite large doses of prednisone, cirrhosis ultimately developed in five patients. The cause of chronic active hepatitis could not be related to azathioprine or methyldopa therapy because there was no perceptible change in the course of liver disease after treatment with these drugs was stopped. Three patients were persistently positive for hepatitis B surface antigen. Isolated instances of granulomatous hepatitis (Mycobacterium kansasii) and of prolonged intrahepatic cholestasis were encountered in patients with chronic active hepatitis. Two patients had acute cytomegalovirus hepatitis. There was one episode each of fulminant herpes simplex hepatitis and severe fatty metamorphosis.
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PMID:Liver disease in renal transplant recipients. 18 93

With the development of simplified methods of bile acid analysis, a new era has drawned in the evaluation of hepatobiliary disease. 1. A total serum bile acid particularly in the postprandial periods is more sensitive than either BSP or ICG for the detection of minimal liver disease and will become a useful screening method. 2. The ratio of chenodeoxycholate to cholate in serum together with the total concentration can often distinguish hepatitis and cirrhosis from intrahepatic and extrahepatic cholestasis with normal liver cell parenchyma. However, in practice this is usually of less value than the total serum bile acid level. 3. Changes in serum bile acids throughout a 24 hour cycle reflect the enterohepatic circulation of bile acids and the capacity of the liver to transport them. These patterns are most useful in judging the severity of cholestasis and response to resin therapy. They also provide new insights into the pathophysiology of bile acid metabolism and excretion in different diseases of the liver.
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PMID:Diagnostic value of serum bile acids. 19 97

The onset of jaundice following a surgical operation sometimes raises difficult problems. It is rarely due to hemolysis, infective hepatitis or decomposated cirrhosis of the liver. One should seek as a routine hepatitis due to halotane. However the most frequent cause is "benign postoperative cholestasis". This variety of jaundice presents in the form of an icterus due to conjugated bilirubine with often a large increase in alkaline phosphatase levels. The ocurse is variable. Almost always due to severe surgical or septic trauma, accompanied by shock and/or anoxia, it raises difficult diagnostic problems. The clinical and physiopathological aspects of benign postoperative cholestasis are recalled. One should remember, above all, that this is not an autonomous clinical entity but the sign of local or general complications which should be sought carefully.
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PMID:[Postoperative medical icterus]. 21 10

Obstructive jaundice, pruritus, and malabsorption developed in twin brothers in infancy. Early liver biopsy specimens showed intracellular and canalicular cholestasis with normal bile ducts. By the age of 3 years, both had cirrhosis and portal hypertension. Each died during the teen years from hepatocellular carcinoma. These brothers represent the tenth reported family with familial cholestatic cirrhosis, and they are the first patients with this syndrome in whom hepatoma developed.
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PMID:Hepatoma in familial cholestatic cirrhosis of childhood: its occurrence in twin brothers. 21 1

The various factors are reviewed which may contribute to the appearance of jaundice in patients with cirrhosis. During the prehepatic phase, hemolysis, spenomegaly and a drop in coagulation factors constitute the main physiopathological mechanisms, whereas intrahepatic cholestasis, alcoholic hepatitis, hepatoma and terminal hepatic insufficiency are the principal mechanisms cited for the hepatic stage. In the posthepatic stage, attention is drawn to the increased frequency of lithiasis in cirrhotic patients and the choledochal lesions seen in chronic pancreatitis.
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PMID:[Icterus and cirrhosis: physiopathology]. 22 39

CT can clearly demonstrate dilation of intra- and extra-hepatic bile ducts due to mechanical obstruction. Note is made that the intrahepatic bile must not necessarily participate in dilation in obstructive jaundice. The cause in 27 cases observed in our institutions was as follows: 16 pancreatic tumors; 1 stone; 2 extrahepatic bile duct obstructions; 4 liver lesions (tumor and cirrhosis) and 4 with cause unknown. Furthermore, CT is helpful in the evaluation of hepatogenic non-obstructive jaundice such as due to primary liver cell carcinoma (hepatoma), metastases to the liver and advanced cirrhosis of the liver. The value of CT in the evaluation of different types of cholestasis is demonstrated by several exemplary cases; and the problems of differential diagnosis are pointed out.
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PMID:[Computerized tomography in the evaluation (author's transl)]. 22 56

Serum glutamic oxaloacetic transaminase (GOT), mitochondrial GOT (GOTm), glutamic-pyruvic transaminase (GPT) and glutamate dehydrogenase activities were determined in 43 healthy controls and in 280 cases of liver diseases. A simplified column chromatographic method coupled with UV assay was employed for separation of GOTm. The activity was measured by following decrease in abosrbance of NADH at 340 nm. The lowest activity of GOTm determined with a coefficient of variation below 10% was 6 mIU/ml. High GOTm activities were found in acute hepatitis (acute stage), subacute hepatitis and primary biliary cirrhosis and were generally associated with high total GOT (GOTt) activities. The activity ratio of GOTm/GOTt varied depending on the stage and severity of liver diseases. The GOTm/GOTt ratio was decreased in acute, fulminant and subacute hepatitides. No significant reduction in the ratio was found in bile duct obstruction, alcoholic liver injury or metastatic liver cancer. Although relatively high GOTm/GOTt ratios were found in some patients with severe hepatic injury, they had no definite association with poor prognosis. These results indicate that the marked elevation in GOTt over GPT in advanced chronic hepatitis, liver cirrhosis and primary hepatoma was mainly due to preferential leakage of cytoplasmic GOT (GOTs).
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PMID:The mechanism of the release of hepatic enzymes in various liver diseases. 1. Alterations in cytoplasmic and mitochondrial enzyme activities in serum. 22 31

Fifty cystic fibrosis (CF) patients, of whom 9 had multilobular cirrhosis, were observed regularly for a period of 3 years and various liver function tests, indicating cytolysis, cholestasis and cellular insufficiency were performed. Immunoglobulin and prothrombin were assayed. In 9 patients with cirrhosis, the tests were generally abnormal. Two distinct biochemical patterns of cirrhosis were distinguished, one clearly cholestatic and the other of a more cellular type. The distinction was made on the basis of the IgA : Transferrin ratio and of gamma-glutamyl-transpeptidase levels. In the non-cirrhotic patients, a temporary increase of cytolysis and cholestasis was observed in 50% of the cases.
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PMID:Additional data on hepatic function tests in cystic fibrosis. 23 31

The enzyme gamma-glutamyl transpeptidase is widely distributed throughout the body, notably kidney, seminal vesicles, pancreas, liver, spleen and brain. Being one of the enzymes of the gamma-glutamyl cycle, it is involved in aminoacid transport, catalysing a transpeptidation reaction between gamma-glutamyl peptides and most common amino acids. Methods of assay of the enzyme are based on its ability also to act on synthetic amides of glutamic acid; kinetic methods monitoring the release of p-nitroaniline from the substrate L-gamma-glutamyl p-nitroanilide are the most satisfactory. In diseases of the liver, the highest levels occur in association with cirrhosis, alcoholism, hepatic secondaries and cholestasis. As the enzyme is present in the endoplasmic reticulum of the hepatocyte, its activity is increased in situations leading to microsomal enzyme induction. Raised levels can also occur in pancreatitis, diabetes, myocardial infarction, congestive cardiac failure, chronic renal failure, cerebrovascular accidents, cerebral tumours and chronic obstructive pulmonary disease. Although the lack of specificity must be recognised, the estimation can be useful in the elucidation of some clearly defined problems arising during investigation of patients with suspected hepatic disease, especially where performed as part of a biochemical profile.
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PMID:Role of gamma-glutamyl transpeptidase activity in the diagnosis of hepatobiliary disease. 24 76


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