Document Detail

Perinatal galactose metabolism.
MedLine Citation:
PMID:  3906069     Owner:  NLM     Status:  MEDLINE    
Galactose is a major nutrient in normal newborn infants and serves as a substrate for energy production and fuel storage and a regulator of carbohydrate assimilation. Inborn errors of galactose metabolism have contributed to our understanding of the potential toxicity of this carbohydrate. In addition to the classic acute manifestations of neonatal galactosemia, long-term follow-up of surviving patients have revealed unusual neurodevelopmental and reproductive problems. Many investigators have suggested that the newborn infant can utilize galactose better than adults and that neonatal galactose assimilation exceeds that of glucose. Galactose may be an excellent substitute for glucose among hyperinsulinemic infants of diabetic mothers or premature infants with glucose intolerance. However, until further investigations are performed to define the role of galactose in newborn nutrition and to determine its potential toxicity, galactose should not be used as the primary carbohydrate in sick newborn infants.
R M Kliegman; J W Sparks
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.; Review    
Journal Detail:
Title:  The Journal of pediatrics     Volume:  107     ISSN:  0022-3476     ISO Abbreviation:  J. Pediatr.     Publication Date:  1985 Dec 
Date Detail:
Created Date:  1986-01-21     Completed Date:  1986-01-21     Revised Date:  2008-11-21    
Medline Journal Info:
Nlm Unique ID:  0375410     Medline TA:  J Pediatr     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  831-41     Citation Subset:  AIM; IM    
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MeSH Terms
Cell Membrane Permeability
Energy Metabolism
Follow-Up Studies
Galactokinase / deficiency
Galactose / metabolism*,  physiology
Galactosemias / genetics*,  metabolism,  therapy
Galactosephosphates / metabolism
Genetic Variation
Infant, Newborn*
Intestinal Absorption
Liver / metabolism
Prenatal Diagnosis
Transferases / deficiency
UDPglucose 4-Epimerase / deficiency
UTP-Hexose-1-Phosphate Uridylyltransferase / deficiency
Grant Support
Reg. No./Substance:
0/Galactosephosphates; 26566-61-0/Galactose; EC 2.-/Transferases; EC; EC Uridylyltransferase; EC 4-Epimerase

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