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biliary atresia/triglyceride
Länken sparas på Urklipp
11 resultat
Effectiveness of Enteral Versus Oral Nutrition With a Medium-Chain Triglyceride Formula to Prevent Malnutrition and Growth Impairment in Infants With Biliary Atresia.
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OBJECTIVE
The aim of this study was to compare the effectiveness of oral (PO) versus enteral nutrition (EN) medium-chain triglyceride (MCT) containing-formula to prevent malnutrition and growth impairment in infants with biliary atresia (BA) waiting for a liver transplant.
METHODS
A total of 15
Medium-chain triglycerides in biliary atresia.
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The use of medium-chain triglycerides in the management of biliary atresia.
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Fat absorption in patients with surgically repaired biliary atresia.
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The degree of fat absorption was studied in 4 patients with unrepaired biliary atresia and 11 with repaired biliary atresia (6 with mild jaundice, 5 with no jaundice). The percent absorption was conspicuously reduced in unrepaired patients (mean 30%, range 21 to 39%), moderately reduced in repaired
Effects of liver transplantation on long-chain polyunsaturated fatty acid status in infants with biliary atresia.
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BACKGROUND
The long-chain polyunsaturated fatty acid (LC-PUFA) status of infants with untreated biliary atresia (BA) is known to be poor and is correlated to the severity of the liver disease. Liver transplantation (LT) markedly increases survival of patients with BA but the extent to which this
Clinical heterogeneity of neonatal intrahepatic cholestasis caused by citrin deficiency: case reports from 16 patients.
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A deficiency of citrin, which is encoded by the SLC25A13 gene, causes both adult-onset type II citrullinemia (CTLN2) and neonatal intrahepatic cholestasis (NICCD). We analyzed 16 patients with NICCD to clarify the clinical features of the disease. Severe intrahepatic cholestasis with fatty liver was
An undescribed subset of neonatal intrahepatic cholestasis associated with multiple hyperaminoacidemia.
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Five patients of cholestatic jaundice and multiple hyperaminoacidemias were uncovered during neonatal mass screening for homocystinuria. All five patients had increased plasma levels of methionine, citrulline, tyrosine, threonine, phenylalanine, lysine and arginine. Compared with those of
Eicosanoid synthesis in children with cholestatic disease.
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The possibility of malabsorption of triglycerides contained in the diets of children with cholestasis suggests a deficiency of essential fatty acids and, therefore, probable effects on eicosanoid metabolism. Children with either biliary atresia (BA) or syndromatic paucity of interlobular bile ducts
Plasma and liver carnitine status of children with chronic liver disease and cirrhosis.
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BACKGROUND
Carnitine is an essential cofactor in the transfer of long-chain fatty acids across the inner mitochondrial membrane for oxidation. As its synthesis is performed in the liver, alterations in carnitine metabolism is expected in liver diseases, especially in cirrhosis.
METHODS
In this
De novo fatty liver due to vascular complications after liver transplantation.
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BACKGROUND
The incidence, risk factors, and natural history of de novo nonalcoholic fatty liver disease (NAFLD) after liver transplantation have not been well described. In this report we examined the risk factors and demographic characteristics of 3 patients.
METHODS
During a 16-year period, we
Clinical pictures of 75 patients with neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD).
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We clarified the clinical features of NICCD (neonatal intrahepatic cholestasis caused by citrin deficiency) by retrospective review of symptoms, management and long-term outcome of 75 patients. The data were generated from questionnaires to paediatricians in charge of the patients. Thirty of the
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