ABSTRACT
When screening for abnormal urinary saccharides with one-dimensional thin-layer chromatography, an unknown component was observed in a position just above that of xylose. This compound was studied by gas chromatography-mass spectrometry and identified as the anhydro sugar beta-glucosan. It was observed in approximately 20% of all urine samples investigated by thin-layer chromatography. Excretory levels varied widely from zero up to 5.3 mmol/l. No correlation with age or disease could be established. The compound was thought to be of exogenous origin.
Subject(s)
Gas Chromatography-Mass Spectrometry , Glucose/analogs & derivatives , Adolescent , Adult , Carbohydrate Metabolism, Inborn Errors/urine , Child , Child, Preschool , Chromatography, Thin Layer , Glycosuria , Humans , InfantABSTRACT
Patients with inherited adenylosuccinase deficiency excrete large quantities of succinyloaminoimidazolecarboxamide riboside (SAICAR) and succinyloadenosine (SAdo). A two-dimensional thin-layer chromatography method for the detection of SAICAR is described. The method consists of isolation of imidazoles with a cation exchange resin; TLC on cellulose plates, solvent I, isopropanol-ammonia 10% (4:1) and II, butanol-acetic acid-water (4:1:1); detection with Pauly reagent. SAICAR gives rise to an isolated spot with a characteristic bluish color. Also a simple one-dimensional thin-layer chromatography method using urine without any pretreatment for screening of high risk populations is given. Four new cases could be diagnosed. Clinical and chemical data, including concentrations of SAICAR and SAdo in urine, plasma and cerebrospinal fluid, determined by cation exchange column chromatography, are presented.
Subject(s)
Adenylosuccinate Lyase/deficiency , Imidazoles/urine , Lyases/deficiency , Purine-Pyrimidine Metabolism, Inborn Errors/diagnosis , Purines/urine , Adenosine/analogs & derivatives , Adenosine/urine , Adolescent , Aminoimidazole Carboxamide/analogs & derivatives , Aminoimidazole Carboxamide/urine , Autoanalysis , Child , Chromatography, Ion Exchange , Chromatography, Thin Layer/methods , Erythrocytes/analysis , Female , Humans , Infant , Male , Purine-Pyrimidine Metabolism, Inborn Errors/blood , Purine-Pyrimidine Metabolism, Inborn Errors/urine , Ribonucleosides/urine , Uric Acid/urineABSTRACT
The evolution of a training and postgraduate course in Anesthesiology to serve two Dutch-speaking regions of Europe is described. Since the program was started in 1978, 520 persons have participated and accounted for 1,267 registrations.
