Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Total parenteral nutrition (TPN) has become a mainstay of modern neonatal care for the increasing population of premature infants who survive their initial pulmonary disease. As with other advances in neonatal therapy, hyperalimentation has associated complications and limitations, primary among them its toxicity to the liver. The basic pathologic lesion is bile cholestasis which is probably multifactorial in etiology. Amino acid solutions, excessive calorie-to-nitrogen ratios, and deficient trace elements and antioxidants have all been implicated in this process. Total parenteral nutrition-cholestasis can progress to portal fibrosis and irreversible cirrhosis if long-term hyperalimentation is required. Most at-risk for this iatrogenic condition are those premature infants less than 1500 g birth weight who are exposed to TPN for longer than two weeks. Enteral feedings providing as little as 10 percent of caloric intake are beneficial, and the prognosis for recovery is good once enteral feedings are established.
 ...
PMID:Hyperalimentation associated hepatotoxicity in the newborn. 156 68

One hundred and four adult patients with cystic fibrosis were evaluated for the presence of liver disease as defined by abnormal liver function tests of six months' duration, histological evidence of fibrosis or cirrhosis, or the presence of portal hypertension, or both. Twenty patients fulfilled these criteria and were evaluated further for the presence of biliary tract abnormalities with biliary scintigraphy using 99Tc diisopropylphenyl-carboxymethyl iminodiacetic acid (DISIDA) and endoscopic retrograde cholangiography. Clearance of 99Tc DISIDA from the liver and biliary tree was diminished at 45 (E45) and 60 (E60) minutes in the patients with liver disease compared with those without liver disease; E45 = 37.8% and 65.8%, p less than 0.01; E60 = 48.2% and 77.5%, p less than 0.01 respectively. Serial analogue images of the extrahepatic biliary tree were consistent with common bile duct obstruction with retention of DISIDA and tapering of the common bile duct in seven of 18 patients with and two of 10 patients without liver disease. Endoscopic retrograde cholangiography showed changes consistent with sclerosing cholangitis, with beading and stricturing of the intrahepatic ducts in 12 of the 14 patients. In all 14 patients, including those in whom biliary scintigraphy had suggested obstruction, no abnormality of the common bile duct was identified. These results indicate that abnormalities of the bile ducts in patients with cystic fibrosis related liver disease are confined to the intrahepatic biliary tree and that common bile duct strictures do not contribute to either the progression or development of liver disease in these patients.
 ...
PMID:Biliary complications of cystic fibrosis. 156 61

Many clinicians subjectively feel that cirrhotic patients frequently have clinical signs of hypermetabolism. However, it is unknown whether hypermetabolism is a constant feature of chronic liver disease, corresponds to liver destruction and repair or is of prognostic value. This article is about resting energy expenditure and substrate oxidation rates in 123 patients with biopsy-proven cirrhosis differing with respect to cause, duration of the disease, biochemical parameters of parenchymal cell damage, cholestasis, liver function, number of complications, clinical staging and nutritional state. Resting energy expenditure varied between 1,090 and 2,300 kcal/day and differed from the predicted values in 70% of the patients. Resting energy expenditure was closely related to fat-free mass, and 52% of the variability could be explained by fat-free mass, age and sex. Of all the patients, 18% were hypermetabolic and 31% were hypometabolic. Hypermetabolism showed no strict association with the cause of cirrhosis, the duration of the disease, liver function, cholestasis, cell damage, clinical staging, blood hemoglobin, plasma thyroid hormone levels or human leukocyte antigens. An increased resting energy expenditure was associated with significant losses of muscle, body cell mass and extracellular mass at unchanged body fat, whereas fat and fat-free mass were increased in hypometabolic patients when compared with normometabolic patients. Lipid oxidation was increased, but glucose oxidation was reduced in nearly all patients with cirrhosis. This was most pronounced at advanced stages of liver disease. Although similar with respect to liver function and clinical staging, 76.2% of hypermetabolic patients had transplants within the observation period, compared with only 16.7% and 8.1% in the normometabolic group and hypometabolic group, respectively. Posttransplantation mortality was independent of pretransplantation resting energy expenditure, but it increased significantly in patients with losses in body cell mass. In conclusion, hypermetabolism is not a constant feature of cirrhosis and results more from extrahepatic than from hepatic factors. It may cause malnutrition and contributes to the clinical outcome of patients with chronic liver disease.
 ...
PMID:Energy expenditure and substrate oxidation in patients with cirrhosis: the impact of cause, clinical staging and nutritional state. 156 18

We present our experience with 5 pediatric patients, 3 males and 2 females, with alpha 1 antitrypsin deficiency. These patients were between the ages of 15 months and 8 years and 4 were of the PI ZZ phenotype and 1 of the PI SZ phenotype. All cases presented with liver disease (neonatal cholestasis, cirrhosis, hepatitis). We comment on the different clinical forms of this entity during childhood, most of which are liver diseases, whereas in the adult it is generally manifest as lung disease.
 ...
PMID:[Alpha-1 antitrypsin deficiency in infancy and childhood]. 158 Apr 32

This study was performed to investigate modifications in the serum bilirubin forms, hepatobiliary enzymes, and some glycoproteic substances in patients during the course of extrahepatic cholestasis (stage A) and following its clinical resolution (stage B). The series consisted of 16 patients: 11 had main bile duct stones; two, benign stenosis of the main bile duct; and three, main bile duct cancer. Cholestasis resolved spontaneously in one case, under endoscopy in two, and following surgery in 13. Five patients with liver cirrhosis and a picture of intrahepatic cholestasis following anesthesia were also investigated. Serum bilirubin forms were measured using van den Bergh's method and the alkaline methanolysis-HPLC procedure; the mono- and di-conjugated forms were considered together in the overall evaluation of the results. The hepatobiliary enzymes (ALP, GGT, and AST) were increased at stage A and significantly decreased at stage B. Similar patterns were observed in total (TB), unconjugated (UB), and conjugated bilirubin (CB) and in the percentage of CB out of TB (% CB). In the majority of patients, % CB at stage B was lower than at stage A, whereas in subjects with a high initial UB value, a different % CB pattern was observed. The direct bilirubin percentage (% DB), on the other hand, had a different pattern, and the variations between stages A and B were not significant. The pathophysiological bilirubin pattern was similar in patients with intrahepatic cholestasis. At stage A, in a number of patients the levels of glycoproteic substances (CA 19-9, TPA and ferritin) were raised, but at stage B they tended to decrease towards the normal range.(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:Alterations in bilirubin metabolism during extra- and intrahepatic cholestasis. 160 Mar 31

A 32 yr-old woman suffering from an unsuspected primary biliary cirrhosis (PBC), completed an uneventual pregnancy. She only experienced diffuse pruritus and subicterus, which misled to an antepartum diagnosis of intrahepatic cholestasis of pregnancy. The most remarkable points of the reported case are: (a) the younger age of onset of PBC; (b) the paucisymptomatic course of PBC; (c) the rarity of PBC-associated pregnancy (only 15 previous instances in 12 patients, from a review of the literature); (d) the uncomplicated course of pregnancy for both the mother and the fetus, which is the exception rather than the rule, in such a condition. The present observation calls our attention to the possible existence of an underlying latent liver cirrhosis in pregnant women with pruritus, jaundice and mild alterations of liver function indexes. This association, which is expected to increase in frequency in the near future, may have important implications in the management of pregnancy.
 ...
PMID:[Uncomplicated pregnancy in patients with unrecognized primary biliary cirrhosis]. 160 28

Alcohol can induce a wide spectrum of histological changes in the liver. Three morphologic patterns of alcoholic liver injury are now generally accepted, i.e. fatty change, alcoholic hepatitis and alcoholic cirrhosis, but a broad array of lesions has been added to this list in recent years. These damage patterns differ considerably in their significance as to indication and diagnostic power of liver biopsies. Liver biopsy is recommended in patients with clinically suspected alcoholic liver disease for diagnostic and prognostic reasons. Moreover, clinicians want to exclude nonalcoholic liver diseases that might otherwise be missed. Alcoholic hepatitis, which is associated with increased morbidity and mortality, has the highest degree of diagnostic specificity in biopsies, because its features are well-defined and are mimicked by a rather small group of other causes. When associated with perivenular and pericellular fibrosis, it may provide prognostic parameters. In contrast, fatty liver, which may be induced by alcohol as well as other etiologies, usually does not need liver biopsy, with some exceptions. It may lead to cholestasis severe enough to mimic obstructive jaundice, or may result in abnormal imaging studies suggesting metastases. Verification of histological findings may be important when these circumstances arise. Cirrhosis is easily verified in biopsies of appropriate quality; however, advanced cirrhosis is a morphologically nonspecific alteration, because cirrhotic tissue patterns converge irrespective of their cause. Liver biopsy may help to identify nonalcoholic liver disease in patients suspected of harboring alcoholic liver disease. In fact, up to 20% of biopsies may show other, potentially treatable disorders, thus extending the indication for liver biopsy in situations of complex clinical and laboratory patterns.
 ...
PMID:[Liver biopsy in suspected alcoholic liver damage]. 162 Dec 36

To study the liver disease of patients with cystic fibrosis, percutaneous liver biopsies were performed in 10 patients with cystic fibrosis aged 6 to 22 yr. Nine of 10 patients had high Shwachman scores, eight had normal serum levels of transaminases. Light-microscopical examination showed steatosis in seven cases and in five slight or moderate inflammatory infiltration. Eight patients showed varying degrees of fibrosis and even cirrhosis. Six patients had bile-duct proliferation and, in one case a bile plug was found. Other signs of cholestasis were not seen. Electron-microscopical investigation showed no specific signs of cholestasis such as ductal plugs or intracellular bile pigments. The canaliculi were not dilated, except in one case. Most patients had bile-duct cells with irregular shapes, protruding into the lumen, and some cases even had necrotic cells. Around the bile ducts and ductules, collagen was deposited and fat-storing cells were a common finding. Our findings do not support the view that cholestasis is the pathogenetic factor in liver disease in cystic fibrosis. A cytotoxic influence on the biliary cells, stimulating collagen deposition, seems more likely.
 ...
PMID:Bile-duct destruction and collagen deposition: a prominent ultrastructural feature of the liver in cystic fibrosis. 163 46

The model of biliary cirrhosis by bile duct ligation was further characterized using PIIIP, 7S-collagen as well as standard enzymes in the serum, prolyl 4-hydroxylase and total hydroxyproline in the liver and light microscopical histology. Five weeks after bile duct ligation there was an increase in total collagen content of the liver to 510% of initial values accompanied by an increase of serum-PIIIP (225%) and 7S-collagen (389%). The time-course of this connective tissue proliferation was biphasic with an initial phase of cholestasis and cellular damage followed by rapidly increasing collagen accumulation. The novel prolyl 4-hydroxylase inhibitor HOE 077 reduced the accumulation of collagen in the liver over 6 weeks by 48%. There were no apparent side effects and treated animals showed a tendency towards less functional impairment. The drug's effects, however, were not dose-dependent between daily doses of two times 2 mg/kg and two times 10 mg/kg. These results emphasize the usefulness of the bile duct ligation model for studies of collagen metabolism. They show HOE 077 to be a promising agent for the inhibition of hepatic fibrosis.
 ...
PMID:Fibrosis of the liver in rats induced by bile duct ligation. Effects of inhibition by prolyl 4-hydroxylase. 166 67

The work deals with a group of 212 patients suffering from various forms of precirrhotic alcoholic liver disease and includes a period of 8.5 years (January 1981-June 1989). At least two liver biopsies were performed in all patients. according to the histological diagnosis, the patients were distributed into 6 subgroups: simple hepatic steatosis--24 cases (11.3%), hepatic fibrosis--40 cases (18.8%), hepatic steatofibrosis--69 cases (32.5%), acute alcoholic hepatitis--18 cases (8.5%), chronic active hepatitis--43 cases (20.3%) and chronic persisting hepatitis--18 cases (8.5%). The assessed histological parameters included: fatty transformation, hepatic fibrosis, inflammatory infiltrate within the lobules and in the portal spaces, hepatocellular necrosis, cholestasis, proliferation of the bile ductules and modification of the lobular architectonic. The work is aimed at pointing out the precirrhotic hepatic histological lesions induced by alcohol and fraught with an increased risk of progression towards liver cirrhosis. The histological sequential examination of alcoholic hepatic lesions confirm the possibility of progression and installation of the cirrhotic stage for a number of these lesions. Liver cirrhosis developed in 44 patients (20.7%) within a period of 3-7 years, on an average 5.5 years. The progression toward cirrhosis occurred in 12 patients (5.7%) with steatofibrosis, in 11 (5.2%) with hepatic fibrosis, in 14 (6.6%) with an intralobular inflammatory infiltrate, in 17 (8%) with hepatocellular necrosis, in 3 (1.4%) with cholestasis, in 5 (2.3%) with proliferation of the bile ductules and in 10 patients (4.7%) with a modification of the lobular architectonic. In addition, cirrhosis was detected in 8 patients (3.8%) with alcoholic hepatitis and in 13 patients (6.1%) with chronic active hepatitis.(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:[The criteria of histological activity and the prognosis in precirrhotic alcoholic hepatopathies]. 167 Jan 14


<< Previous 1 2 3 4 5 6 7 8 9 10