In diabetic patients with hyperlipoproteinemia type IV the monohexosyl ceramide concentration in blood plasma is significantly elevated. This augmentation can be attributed to an increased monohexosyl ceramide content of the BLDL plasma fraction. In contrast, the di-, tri-, the tetrahexosyl ceramide levels remain within normal limits. In normolipidemic diabetics of comparable age, sex, and weight classes and of comparable metabolic control no elevations of glycolipid fractions could be found. However, patients with primary hyperlipoproteinemia type IV show an increase of monohexosyl ceramide concentrations in blood plasma. Therefore, the augmentation of monohexosyl ceramide levels in plasma of hyperlipidemic diabetic patients seems rather a metabolic consequence of hyperlipoproteinemia than of diabetes per se. A clearcut explantation for the monohexosyl ceramide elevations in the studied groups cannot be given at present time.
With a new formulation, which made intravenous infusion of triamterene (TA) possible, plasma levels and urinary excretion rates of TA and its main metabolite (OH-TA-ester) were measured in a randomized, cross-over trial in 6 healthy volunteers given triamterene 10 mg i.v. and 50 mg p.o. TA and OH-TA-ester were determined by densitometric measurement of native fluorescence after thin layer chromatography. Distribution volumes of the central compartment of TA and OH-TA-ester were 1.49 l/kg and 0.11 l/kg, respectively. Terminal half-lives were 255 min for TA and 188 min for OH-TA-ester after i.v. administration. For TA total plasma clearance was 4.5 l/min and renal plasma clearance 0.22 l/kg. The formation of OH-TA-ester was very rapid and the concentration of the metabolite exceeded that of TA at all times. After i.v. administration the urinary recovery of TA and OH-TA-ester was 4.4% and 50.9%, respectively. The bioavailability of TA was 52%, corresponding to absorption of 83%. TA is partly eliminated by a first-pass-effect. The main metabolite of TA is OH-TA-ester, which is pharmacologically active.
A 20-year-old man suffering from Crohn's disease developed coma and generalized seizures following ileocecal resection. During postoperative parenteral feeding he received xylitol in an unusually high concentration. CT examinations a few days before death showed intense hypodensity and swelling of brainstem and basal ganglia and increasing triventricular dilatation. Autopsy revealed, mainly in the brainstem and cerebellum, a destruction of intracerebral, intracerebellar and leptomeningeal vessel walls by birefringent crystals (probably calcium oxalate), an early inflammatory reaction and severe brain edema with final tonsillar herniation. The same crystalloid deposits were found in the kidneys.
A method is described for the quantitative isolation and estimation of the four glycosphingolipid fractions from 10 ml of human plasma. The procedure consists of acetylation of the total lipids after extraction, separation of acetylated glycosphingolipids from non-glycolipids on a Florisil column, deacetylation, dialysis in water, separation of the single fractions by thin-layer chromatography on silica gel, and quantitative analysis of each glycosphingolipid by sulfuric acid-orcinol reagent, carried out in the presence of silica gel. The concentrations of the four glycosphingolipids in human plasma derived from a group of 23 healthy subjects are presented. The procedure described is sufficiently sensitive for clinical investigation. Quantitative Bestimmung der Glycosphingolipide im menschlichen BlutplasmaZusammenfassung: Über eine Methode zur quantitativen Isolierung und Bestimmung der vier neutralen Glycosphingolipidfraktionen aus 10 ml menschlichem Blutplasma wird berichtet. Das Verfahren besteht aus: Acetylierung der extrahierten Gesamtlipide, Abtrennung der acetylierten Glycosphingolipide durch Säulenchromatographie an Florisil, Deacetylierung, Dialyse gegen Wasser, Auftrennung der einzelnen Fraktionen durch Dünnschichtchromatographie auf Kieselgelplatten und quantitative Bestimmung der verschiedenen Glycosphingolipide im Schwefelsäure-Orcinol-Test in Gegenwart von Kieselgel. Die ermittelten Konzentrationen der vier Glycosphingolipide in einem Kollektiv von 23 gesunden Personen werden mitgeteilt. Das beschriebene Verfahren ist empfindlich und geeignet für klinische Untersuchungen.
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