Impact on Isoleucine and Valine Supplementation When Decreasing Use of Medical Food in the Nutritional Management of Methylmalonic Acidemia.

Abstract

BACKGROUND:

Methylmalonic acidemia (MMA) is an autosomal recessive disorder treated with precursor-free medical food while limiting natural protein. This retrospective chart review was to determine if there was a relationship between medical food, valine (VAL) and/or isoleucine (ILE) supplementation, total protein intake, and plasma amino acid profiles. Methods: A chart review, of patients aged 31 days or older with MMA treated with dietary intervention and supplementation of VAL and/or ILE and followed at the Children's Hospital Colorado Inherited Metabolic Diseases Clinic. Dietary prescriptions and plasma amino acid concentrations were obtained at multiple time points.

RESULTS:

Baseline mean total protein intake for five patients was 198% of Recommended Dietary Allowance (RDA) with 107% natural protein and 91% medical food. Following intervention, total protein intake (p = 0.0357), protein from medical food (p = 0.0142), and leucine (LEU) from medical food (p = 0.0276) were lower, with no significant change in natural protein intake (p = 0.2036). At baseline, 80% of patients received VAL supplementation and 100% received ILE supplementation. After intervention, only one of the cohort remained on supplementation. There was no statistically significant difference in plasma propiogenic amino acid concentrations.

CONCLUSIONS:

Decreased intake of LEU from medical food allowed for discontinuation of amino acid supplementation, while meeting the RDA for protein.

• Authors: Laurie E Bernstein, Casey Burns, Morgan Drumm, Sommer Gaughan, Melissa Sailer, Peter R Baker 2nd
• Journal: Nutrients (Nutrients) Vol. 12 Issue 2 (Feb 13 2020) ISSN: 2072-6643 [Electronic] Switzerland
• PMID: 32069872 (Publication Type: Evaluation Study, Journal Article)

Chemical References

• Dietary Proteins
• Isoleucine
• Valine

Topics

• Amino Acid Metabolism, Inborn Errors (metabolism, therapy)
• Dietary Proteins (administration & dosage)
• Dietary Supplements
• Enteral Nutrition (methods)
• Female
• Humans
• Infant
• Isoleucine (administration & dosage)
• Male
• Retrospective Studies
• Treatment Outcome
• Valine (administration & dosage)