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A preterm infant with secondary carnitine deficiency due to MCT formula--effective treatment of L-carnitine
A Ishida1, A Goto, Y Takahashi
1Department of Pediatrics, Akita University School of Medicine, Japan.
Insights
Preterm infants on medium-chain triglyceride (MCT) formula can develop carnitine deficiency. This condition can lead to liver dysfunction and elevated creatine kinase (CK), requiring L-carnitine supplementation.
Area of Science:
- Neonatology
- Nutritional Science
- Biochemistry
Background:
- Preterm infants often require specialized nutritional support.
- Medium-chain triglyceride (MCT) formulas are used to manage fat malabsorption.
- Carnitine is essential for fatty acid metabolism.
Observation:
- A preterm infant fed an MCT-rich formula developed steatorrhea.
- The infant later presented with hepatomegaly and elevated liver enzymes (AST, ALT) and creatine kinase (CK).
- Liver biopsy confirmed liver damage.
Findings:
- The infant exhibited low serum carnitine levels.
- High urinary losses of acylcarnitine and dicarboxylic aciduria were observed.
- These findings indicated carnitine deficiency exacerbated by increased urinary excretion.
Implications:
- Prolonged use of MCT formula in preterm infants may increase the risk of carnitine deficiency.
- Monitoring carnitine levels and urinary metabolites is crucial in preterm infants on long-term MCT formula.
- L-carnitine supplementation can effectively treat MCT-induced carnitine deficiency in this population.
Abstract:
We report a preterm infant who was prescribed an MCT formula and subsequently developed carnitine deficiency with liver dysfunction and an elevation of serum CK level. A male infant who had been born at 24 weeks' gestation with birth weight 799 g, was fed with an MCT formula containing 76.8% of all kinds of lipids, because of his steatorrhea after the 30th day. On the 100th day, he was noted hepatomegaly and elevation of serum levels of AST, ALT and CK. The needle biopsy of the liver indicated the existence of the liver damage. He showed low serum carnitine with high urinary loss of acylcarnitine and dicarboxylic aciduria. Administration of L-carnitine was an effective treatment. The carnitine deficiency might be exaggerated by an increased urinary loss of acylcarnitine. We should be cautious of the risk of carnitine deficiency in preterm infants during prolonged use of MCT formula.