Amino Acid Supplementation

Some nutritionists may supplement your child's diet with one or more of the offending amino acids: valine, isoleucine, methionine and threonine.   It can be difficult to locate a source for the amino acids and may require a compounding pharmacy or hospital pharmacy.  The following suppliers are options the amino acids:

• Spectrum Chemicals
• Vitaflo USA

  Isoleucine Amino Acid Supplement

  Valine Amino Acid Supplement

   

L-Carnitine Supplementation

L-Carnitine is often prescribed.  The following medical articles explain how carnitine binds with propionic acid and leaves the body through the urine:

• L-carnitine enhances excretion of propionyl coenzyme A as propionylcarnitine in propionic acidemia. C R Roe, D S Millingoton, D A Maltby, T P Bohan, and C L Hoppel. l
• Propionylcarnitine excretion in propionic and methylmalonic acidurias: a cause of carnitine deficiency.
  Di Donato S, Rimoldi M, Garavaglia B, Uziel G.

The following companies distribute L-Carnitine:

• Sigma Tau Pharmaceuticals
• Rising Pharmaceuticals, Inc.