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Citrin plays a role in the transfer of NADH-reducing equivalent from cytosol to mitochondria as part of the malate-aspartate shuttle in liver. Citrin deficiency may cause an impairment of glycolysis due to an increase in the cytosolic NADH/NAD ratio leading to an energy shortage in the liver.
Essential fatty acid (EFA) deficiency during cholestasis is mainly due to malabsorption of dietary EFA (23). Theoretically, dietary phospholipids (PL) may have a higher bioavailability than dietary triglycerides (TG) during cholestasis. We developed murine models for EFA deficiency (EFAD) with and
OBJECTIVE
Lipoprotein metabolism in FIC1 deficiency due to ATP8B1 mutations has never been studied sufficiently. This study was performed to investigate the detailed lipoprotein metabolism in benign recurrent intrahepatic cholestasis (BRIC) caused by FIC1 deficiency.
METHODS
Lipoprotein profile and
A randomized, double blind prospective study made on surgical patients who required parenteral nutrition during a 10-day period, with complete fasting. The patients were required to show a normal hepatic function measured by gamma-GT, alkaline phosphatase (FA), normal bilirubin and ALT. The
BACKGROUND
This study aimed to compare the effect of 2 lipid emulsions (LEs), a medium-chain triglyceride (MCT)/ω-3-polyunsaturated fatty acid (PUFA)-containing LE and a soybean-based LE, on the incidence of neonatal cholestasis, bronchopulmonary dysplasia (BPD), and lipid profile of preterm
Former studies have shown that curcumin, which can be extracted from different Curcuma species, is able to stimulate bile flow and to reduce hypercholesterolemia. We investigated in a subchronic bile fistula model the ability of curcumin to reduce cyclosporine-induced cholestasis and
D-Alpha-tocopheryl polyethylene glycol 1000 succinate (Tocofersolan, Vedrop), has been developed in Europe to provide an orally bioavailable source of vitamin E in children with cholestasis. The aim was to analyze the safety/efficacy of Vedrop in a large group of children with chronic
Neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD) is an autosomal recessive disease caused by the dysfunction of citrin, an aspartate/glutamate carrier encoded by the SLC25A13 gene. Considerable progress has been made on the diagnosis and treatment of NICCD, but its clinical and
The authors report the main morphological and functional alterations of the liver during the course of pregnancy. The size of the organ does not change and there is a slight (20%) reduction of hepatic flow. Hepatic function is partially modified in view of the following factors: reduced protein
BACKGROUND
Cholangiocarcinoma is an invasive biliary malignancy with poor prognosis. Diagnostic accuracy of conventional methods is low which is mainly due to the specific anatomy of the disease. The aim of this study was to evaluate the diagnostic value of biochemical profile and tumor marker of
Neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD) is a kind of inborn errors of metabolism, with the main clinic manifestations of jaundice, hepatomegaly, and abnormal liver function indices. As a mitochondrial solute carrier protein, citrin plays important roles in aerobic
OBJECTIVE
To identify the influence of the increased level of serum cholyglycine (CG) on lipid metabolism and hemorrheology in patients with intrahepatic cholestasis during pregnancy (ICP).
METHODS
The concentrations of serum CG, total cholesterol (CH), triglycerides (TG), high density
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
OBJECTIVE
Intrahepatic cholestasis of pregnancy (ICP) is associated with increased perinatal mortality and morbidity. Alpha-hydroxybutyrate dehydrogenase (alpha-HBDH) is an enzyme that originates in the cytoplasm of hepatocytes and can be detected in the serum. The aim of this study was to determine
OBJECTIVE
To analyse lipoprotein lipase (LPL) expression and lipid levels in placenta and plasma of patients with intrahepatic cholestasis of pregnancy (ICP) and normal pregnancies.
METHODS
This prospective study included 30 patients with ICP and 30 gestational-age-matched pregnancies without any