11 rezultātiem
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
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
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
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
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
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
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
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
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