DOSAGE AND ADMINISTRATION:
To be determined by the clinician or dietitian and is dependent on the age, body weight, and medical condition of the patient.
For routine outpatient management, plasma valine and isoleucine levels should be maintained between 200 and 400 μmol/L (1).
Supplementation with valine and isoleucine is particularly important to support protein synthesis and facilitate leucine clearance (2). Valine, in particular, has a low affinity for the LAT1 transporter at the blood-brain barrier and is especially vulnerable to competitive inhibition by leucine (3), therefore adequate supplementation is necessary. If either valine or isoleucine falls below the treatment range, a suggested starting dose of 50–100 mg/day should be given (4).
When switching from powders that require dissolving in liquid, a 4g Valine packet is equivalent to 1ml of Meta Healthcare Ready to Use (RTU) liquid Valine and a 4g Isoleucine packet is equivalent to 2ml of Meta Healthcare RTU liquid Isoleucine formulation.
(2) Single amino acid supplementation in aminoacidopathies: a systematic review – PubMed
(4) Hassan SA, Gupta V. Maple Syrup Urine Disease. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 [cited 2025 Apr 14]. Available from: http://www.ncbi.nlm.nih.gov/books/NBK557773/



