cholestasis/triglyceride

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Treatment with lactose (galactose)-restricted and medium-chain triglyceride-supplemented formula for neonatal intrahepatic cholestasis caused by citrin deficiency.

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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.

Treatment of EFA deficiency with dietary triglycerides or phospholipids in a murine model of extrahepatic cholestasis.

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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

Depletion of high-density lipoprotein and appearance of triglyceride-rich low-density lipoprotein in a Japanese patient with FIC1 deficiency manifesting benign recurrent intrahepatic cholestasis.

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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 comparative study of hepatic cholestasis after infusion of long chain triglycerides and a mixture of medium and long chain triglycerides].

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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

Cholestasis, bronchopulmonary dysplasia, and lipid profile in preterm infants receiving MCT/ω-3-PUFA-containing or soybean-based lipid emulsions.

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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

Effects of curcumin on cyclosporine-induced cholestasis and hypercholesterolemia and on cyclosporine metabolism in the rat.

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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

Oral Tocofersolan Corrects or Prevents Vitamin E Deficiency in Children With Chronic Cholestasis.

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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

Multiple ovarian antral follicles in a preterm infant with neonatal intrahepatic cholestasis caused by citrin deficiency: a clinical, genetic and transcriptional analysis.

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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

[Cholestasis in pregnancy].

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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

Biochemical profile of bile fluid in patients with malignant cholestasis in comparison with cholestasis due to gall stone.

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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

[A difficult and complicated case study: neonatal intrahepatic cholestasis caused by citrin deficiency].

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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

[The changes of lipid metabolism and hemorrheology in intrahepatic cholestasis during pregnancy].

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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

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

Alpha-hydroxybutyrate dehydrogenase activity in intrahepatic cholestasis of pregnancy.

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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

Lipoprotein lipase and lipid profiles in plasma and placenta from normal pregnancies compared with patients with intrahepatic cholestasis of pregnancy.

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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

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