Frans Stellaard

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BACKGROUND & AIMS Progressive familial intrahepatic cholestasis (PFIC), an inherited liver disease of childhood, is characterized by cholestasis and either normal or increased serum gamma-glutamyltransferase activity. Patients with normal gamma-glutamyltransferase activity have mutations of the FIC1 locus on chromosome 18q21 or mutations of the BSEP gene on(More)
A sensitive and selective analytical technique is described for the determination ofN-acetylaspartic acid in body fluids using stable isotope dilution in combination with positive chemical ionization mass spectrometry with selected ion monitoring. Control mean and ranges have been established: in urine 19.5 and 6.6–35.4 µmol/mmol creat.; in plasma 0.44 and(More)
This review aims to present an overview of the application of stable isotope technology in clinical pharmacology. Three main categories of stable isotope technology can be distinguished in clinical pharmacology. Firstly, it is applied in the assessment of drug pharmacology to determine the pharmacokinetic profile or mode of action of a drug substance.(More)
Sir: Dihydropyrimidine dehydrogenase (DHPD)-deficiency (McKusick 27427) is an autosomal recessively inherited defect of pyrimidine catabolism [1, 2]. Although the clinical presentat ion shows considerable variation, neurological abnormalities are frequent. We recently diagnosed a patient with a relatively severe phenotype, presenting with microcephaly and(More)
BACKGROUND Resistant starch sources, which are only partially digested in the small intestine, can be used to increase colonic availability of short-chain fatty acids. OBJECTIVE To study the characteristics of the fermentation of resistant starch, the digestion of resistant starch in the small intestine has to be quantified. We compared the metabolic(More)
OBJECTIVE To assess the accuracy of six commonly used diagnostic tests for Helicobacter pylori in a prospective study without using any specific test as the gold standard (the patient was regarded as H. pylori-infected if two or more tests, whatever their nature, were positive). METHODS In 105 outpatients undergoing upper GI endoscopy, 62 without(More)
BACKGROUND High-fat diets promote hepatic lipid accumulation. Paradoxically, these diets also induce lipogenic gene expression in rodent liver. Whether high expression of these genes actually results in an increased flux through the de novo lipogenic pathway in vivo has not been demonstrated. METHODOLOGY/PRINCIPAL FINDINGS To interrogate this apparent(More)
Effects of 17alpha-ethinylestradiol (EE) on the neutral and acidic biosynthetic pathways of bile salt (BS) synthesis were evaluated in rats with an intact enterohepatic circulation and in rats with long-term bile diversion to induce BS synthesis. For this purpose, bile salt pool composition, synthesis of individual BS in vivo, hepatic activities, and(More)
UNLABELLED Diabetes is characterized by high blood glucose levels and dyslipidemia. Bile salt sequestration has been found to improve both plasma glycemic control and cholesterol profiles in diabetic patients. Yet bile salt sequestration is also known to affect triglyceride (TG) metabolism, possibly through signaling pathways involving farnesoid X receptor(More)
Impaired intestinal barrier function is observed in type 1 diabetes patients and animal models of the disease. Exposure to diabetogenic antigens from the intestinal milieu due to a compromised intestinal barrier is considered essential for induction of the autoimmune process leading to type 1 diabetes. Since a hydrolysed casein (HC) diet prevents autoimmune(More)