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Carnitine metabolites in infants with cystic fibrosis: a prospective study
Author(s) -
LloydStill John D,
Powers Catherine A,
Wessel Hans U
Publication year - 1993
Publication title -
acta pædiatrica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 115
eISSN - 1651-2227
pISSN - 0803-5253
DOI - 10.1111/j.1651-2227.1993.tb12626.x
Subject(s) - carnitine , cystic fibrosis , medicine , endocrinology , cord blood , long chain , gastroenterology , urinary system , metabolism , prospective cohort study , fatty acid metabolism , chemistry , polymer science
Acylcarnitine is low in cord blood in patients with cystic fibrosis, suggesting that fatty acid metabolism is disturbed in utcro. 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.