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Carnitine metabolites in infants with cystic fibrosis: a prospective study

J D Lloyd-Still1, C A Powers, H U Wessel

  • 1Cystic Fibrosis Center, Children's Memorial Hospital, Northwestern University, Chicago, Illinois 60614.

Acta Paediatrica (Oslo, Norway : 1992)
|February 1, 1993
PubMed

Insights

Infants with cystic fibrosis have low acylcarnitine levels, indicating disturbed fatty acid metabolism. Nutritional management improved some carnitine levels, but others remained low, suggesting altered carnitine regulation in cystic fibrosis.

Area of Science:

  • Biochemistry
  • Pediatric Nutrition
  • Metabolic Disorders

Background:

  • Acylcarnitine deficiency in cord blood suggests in utero fatty acid metabolism disturbance in cystic fibrosis (CF).
  • Carnitine is essential for fatty acid transport and energy production.

Purpose of the Study:

  • To prospectively measure carnitine metabolites in infants with CF.
  • To assess the impact of nutritional management on carnitine levels in CF.

Main Methods:

  • Prospective study of 23 infants with newly diagnosed CF.
  • Measurement of plasma and urinary carnitine metabolites (total, free, short-chain, long-chain).
  • Comparison with 48 healthy controls over 6-12 months and up to 3 years of age.

Main Results:

  • Significantly lower plasma total, free, and long-chain carnitines at diagnosis in CF infants compared to controls.
  • Nutritional management normalized total and free carnitine, but not short- and long-chain acylcarnitines.
  • All carnitine metabolites remained lower than controls by age three, with increased urinary excretion.

Conclusions:

  • CF infants exhibit persistent carnitine metabolite deficiencies despite dietary intervention.
  • Increased urinary excretion suggests disturbed carnitine regulation and utilization in CF.
  • The long-term physiological significance of these carnitine alterations in CF requires further investigation.

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