Urinary excretion of pentose phosphate pathway-associated polyols in early postnatal life.
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.


OBJECTIVE
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.
Koy
Anne
A
Department of General Pediatrics, University Children's Hospital, Düsseldorf, Germany. anne.koy@med.uni-duesseldorf.de
Waldhaus
Anne
A
Hammen
Hans-Werner
HW
Wendel
Udo
U
Mayatepek
Ertan
E
Schadewaldt
Peter
P
D000367:Age Factors; D002648:Child; D002675:Child, Preschool; D005260:Female; D005865:Gestational Age; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D010427:Pentose Phosphate Pathway / Q000502:physiology*; D013402:Sugar Alcohols / Q000652:urine*; D016482:Urinalysis
D013402:Sugar Alcohols