Isovaleric Aciduria is an inherited metabolic disease related to the metabolism of the amino acid leucine.
Amino acids are the constituent units of proteins and are therefore daily taken as part of one’s diet. Individuals with Isovaleric Aciduria should follow a diet that does not contain the amino acid leucine, in order to minimize isovaleric acid formation.
Given the impossibility of excluding this amino acid from natural proteins, the patient, in general, should follow a protein-free diet, supplemented with adequate protein substitutes (without leucine).
PIAM, always attentive to the problems related to rare or low-prevalence diseases, offers a diversified range of specific protein substitutes for the correct nutritional management of patients affected by Isovaleric Aciduria, providing tailor-made solutions for all ages, from infancy to adulthood.