To date, two new defects in the pentose phosphate pathway have been identified in patients with abnormalities in their polyol profiles. Some of them presented with neurological symptoms of so far unknown aetiology. The pathophysiological role of polyols, e.g. in the brain, is relatively unknown. We tested the neurotoxicity of polyols using a 'neurochip' model. After exposure of cortical rat neurons to D-arabitol and ribitol in increasing concentrations up to 10 mmol/L, the electrophysiological activity was measured. No acute effect on the spontaneous network activity of cortical neurons was observed. We speculate that polyols have only secondary effects on brain dysfunction.
Background: Two new inborn errors in the pentose phosphate pathway have been described: ribose-5-isomerase deficiency and transaldolase deficiency. These defects are characterized by accumulation of specific polyols in body fluids. Little is known about human polyol metabolism, but there are indications for a physiological role primarily during early development. Objectives: The objective of this study was to evaluate the urinary excretion of polyols in neonates with special interest on a possible impact of the grade of maturity. For comparison, urinary polyol excretion in older children was also studied. Methods: Urine samples of 40 neonates born between gestational week 25 and 41 were analyzed for the excretion of pentose phosphate pathway-associated polyols (erythritol, D-arabitol, ribitol, xylitol). These metabolites were also quantified in urine obtained from 77 children aged 4 weeks to 10 years. Results: The results show high urinary polyol excretions after birth independent of the week of gestation. During the first months of life, the concentrations decreased exponentially and reached a fairly stable steady state thereafter. Conclusions: Urinary excretion of polyols shows an age dependency with highest concentrations postnatally independent of the grade of maturity. These findings suggest a possible connection between the formation of pentose phosphate pathway-associated polyols and fetal development.
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