关键词: 遗传代谢病, 蛹油, 不饱和脂肪酸, α-亚麻酸, 营养素

Abstract: Inherited metabolic disorders have complex clinical manifestations with high rate of disability and mortality. The symptoms of the patients are usually non-specific，which results in difficult diagnosis. Biochemical and genetic examination should be performed as early as possible to confirm the diagnosis and save time for treatment. Dietary and nutritional interventions are important measures for the prevention and treatment of inherited metabolic disorders.Unsaturated fatty acids are essential nutrients in neurodevelopment and functional maintenance. Conventional diets cannot provide enough unsaturated fatty acids for children. Supplementation of unsaturated fatty acids is necessary as a main or supportive treatment. Bombyx mori oil is rich in α-linolenic acid and can be used as medicine and food. Traditional medical research has proved that bombyx mori oil has good nutritional and medicinal value，having the effect of anti-inflammation，anti-allergy，anti-cancer，lowering blood sugar，regulating lipid，scavenging free radicals，antioxidation，and improving memory and other efficacy. This review summarizes the characteristics of bombyx mori oil and α-linolenic acid to provides reference for the application of bombyx mori oil in the treatment of inherited metabolic disorders from the perspective of nutritional intervention.

Key words: inherited metabolic disorders, bombyx mori oil, α-linolenic acid, unsaturated fatty acid, nutrient