When the human organism, due to a genetic defect, is unable to correctly metabolize certain types of fatty substances, we are dealing with a congenital lipid metabolism disorder.
The early diagnosis of some of these conditions, classified as rare diseases, has recently been improved following the introduction in Italy of the extended screening of the neonatal population.
BIOS, always attentive to the particular needs of patients with rare or low-prevalence diseases, is able to offer nutritional support therapies for people suffering from certain disorders of lipid metabolism.
Related diseases
Fatty acid oxidation disorders
Inborn disorders of lipid metabolism that reduce the body’s ability to convert fat into energy are normally defined as fatty acid oxidation disorders and fall within the large family of Hereditary Metabolic Diseases (HMDs).
Fatty acids are one of the main energy substrates for the body. When these are not efficiently metabolised to produce energy, there may be various problems for the body characterized by complex systemic clinical patterns and organ damage.
The long-term treatment of patients with these diseases generally involves:
- avoiding prolonged fasting
- avoiding excessive muscle strain
- establishing a diet therapy based on small and frequent meals, rich in complex carbohydrates and low in fat.
- limiting the intake of long-chain fatty acids (LCT) and increasing the intake of medium-chain fatty acids (MCT). This dietary approach allows an adequate caloric intake to be reached, which, especially in children, would be impossible with only proteins and carbohydrates.
The treatment is, however, customized according to the needs of the individual patient.
BIOS, always attentive to the particular needs of patients suffering from rare or low-prevalence diseases, is able to offer nutritional support therapies for patients suffering from fatty acid oxidation disorders.