Isovaleric acidemia/aciduria is a hereditary metabolic disease from the group of organic acids caused by a mutation of the IVD gene encoding the enzyme isovaleryl-CoA dehydrogenase, which leads to deficiency or absence of its activity and disruption of the breakdown of leucine.
Basic principles of diet therapy:
– protein restriction of natural foods
– mandatory compensation of protein deficiency through the use of specialized mixtures based on amino acids without leucine
– ensuring sufficient energy value of the diet
– providing sufficient fluids
– fractional feedings, without long breaks
– Teaching parents the rules of organizing diet therapy
The Ministry of Health of the Russian Federation approved the standard of medical care for children with IVA, which includes the use of specialized leucine-free medical nutrition products, as well as solutions for parenteral nutrition.