Carnitine metabolites in infants with cystic fibrosis: a prospective study.
Acylcarnitine is low in cord blood in patients with cystic fibrosis, suggesting that fatty acid metabolism is disturbed in utero. Carnitine metabolites (total, free, short- and long-chain acylcarnitine) were measured prospectively in 23 newly diagnosed infants with cystic fibrosis treated with a carnitine-containing, predigested formula for 6-12 months. Total (p < 0.002), free (p < 0.004), and long-chain (p < 0.001) plasma concentrations of carnitines were significantly less than controls (n = 48) at diagnosis. Total and free concentrations were corrected with nutritional management, whereas short- and long-chain acylcarnitines remained unchanged. By three years of age all plasma concentrations of carnitine metabolites were significantly less than controls despite a carnitine-containing diet. Urinary carnitine metabolites were increased at diagnosis and follow-up. The physiological significance of these observations in cystic fibrosis is unknown, but could be compatible with disturbed regulatory control with resultant increased utilization.
Lloyd-Still
J D
JD
Cystic Fibrosis Center, Children's Memorial Hospital, Northwestern University, Chicago, Illinois 60614.
Powers
C A
CA
Wessel
H U
HU
D000108:Acetylcarnitine / Q000378:metabolism; D000367:Age Factors; D002331:Carnitine / Q000008:administration & dosage / Q000378:metabolism*; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000188:drug therapy / Q000378:metabolism*; D004734:Energy Metabolism; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D011446:Prospective Studies
D000108:Acetylcarnitine; D002331:Carnitine