Experience of Using Lipano in Children with Fatty Acid Oxidation Disorders

Disorders of mitochondrial beta-oxidation of fatty acids - a group of rare hereditary metabolic disorders. Deficiency of long-chain 3-hydroxyacyl CoA dehydrogenase of fatty acids (LCHADD) is one of the most frequent in Europe inherited metabolic disorders from the group of defects of mitochondrial beta-oxidation of fatty acids. This disease is characterized by pathology of the liver, heart and skeletal muscle, frequent metabolic crises, and high mortality in infancy. This article discusses the basic principles of diet therapy for long-chain fatty acid oxidation disorders and the experience of using the specialized therapeutic food product «Lipano» (Kanso, Italy) in children with a confirmed hereditary disorder of mitochondrial beta-oxidation of fatty acids.

hereditary metabolic diseases, disorders of mitochondrial beta-oxidation of fatty acids, insufficiency of long-chain 3-hydroxy acetyl-CoA fatty acid dehydrogenase (LCHAD), insufficiency of very long-chain acetyl-CoA fatty acid dehydrogenase (VLCAD), carnitine palmitoyl transferase I (CPT I), carnitine palmitoyl transferase II (CPT II), specialized therapeutic nutrition products

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