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Carnitine metabolites in infants with cystic fibrosis
1Cystic Fibrosis Center, Children's Memorial Hospital, Northwestern University, Chicago, Illinois 60614.
Insights
Carnitine levels in infants with cystic fibrosis (CF) were studied. Supplementation normalized carnitine status, suggesting no primary carnitine metabolism defect in CF.
Area of Science:
- Biochemistry
- Pediatrics
- Metabolic Disorders
Background:
- Infants with cystic fibrosis (CF) may have altered carnitine metabolism.
- Previous studies indicated decreased acylcarnitine levels in CF infant cord blood.
Purpose of the Study:
- To investigate carnitine status in newly diagnosed infants with CF.
- To determine if carnitine levels normalize after treatment and supplementation.
Main Methods:
- Measured blood and urine carnitine metabolites in 15 infants with newly diagnosed CF.
- Monitored carnitine levels for one year during treatment with specialized formula and carnitine supplements.
Main Results:
- No consistent carnitine abnormalities were found in newly diagnosed CF infants.
- Carnitine levels normalized within one year of age after therapeutic intervention.
Conclusions:
- The findings do not support a primary carnitine metabolism disorder in cystic fibrosis.
- Carnitine supplementation may be beneficial in managing metabolic status in CF infants.
Abstract:
Decreased acylcarnitine levels have been found in cord blood of CF infants compared to siblings and controls. We therefore measured carnitine metabolites in blood and urine in 15 newly diagnosed (average age 4 mos) CF infants and followed the levels for one year. No consistent abnormality in carnitine status was detected in newly diagnosed infants; levels normalized at one year after therapy with predigested formula containing carnitine supplements. This data does not provide support for a primary abnormality of carnitine metabolism in CF.