32 Intestinal Absorption Of Pteridines In Childhood View Full Text


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Article Info

DATE

1988-08

AUTHORS

D Turck, J L Dhondt, J M Hayte, J P Farriaux

ABSTRACT

Oral tetrahydrobiopterin (THB) load has been recommended for the recognition of THB deficiency among hyperphenylalaninemic infants. In some patients with dihydropteridine reductase (DHPR) deficiency, the test has been reported to be ineffective. To explore such a resistance to THB, intestinal absorption of pteridines was investigated. In 10 control children the maximum biopterin (B) serum value was observed 6 hours after oral administration of 5 mg/kg THB. A wide range of B increase (14.6-190.5 nmol/l, mean : 73.2 nmol/l) was noted, suggesting a great variation in the intestinal absorption of THB. A constant rate intestinal perfusion study using a double lumen tube and polyethylene glycol 4000 as non absorbable marker was performed in 8 control infants and 1 adult. Pteridines were separated after acid and alcaline oxydation by ion-exchange HPLC chromatography. THB, B, and pterin mean absorption rates were as follows : THB = 14% (n=9), B=3% (n=3), pterin = 80 % (n=3). These results show a very low intestinal absorption of THB and B in man, and suggest the limiting role of the lateral chain 6-dihydroxypropyl, present in THB and B, and absent in pterin. They could explain the resistance to TUB during the oral loading test in DHPR deficient patients. More... »

PAGES

266

Identifiers

URI

http://scigraph.springernature.com/pub.10.1203/00006450-198808000-00058

DOI

http://dx.doi.org/10.1203/00006450-198808000-00058

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1006143973


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