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Patients with long-chain fatty acid oxidation defect (LCFAOD) cannot tolerate fasting and are restricted in their physical activity, hence their increased risk of obesity. Experts therefore advise avoidance of catabolic situations and discourage weight reduction in these patients.Two patients with late-diagnosed LCFAOD undergoing treatment at two academic centers successfully lost weight under supervision of a metabolic dietitian. Patient 1 (male, 47 years) diagnosed with CPT 2 deficiency lost 10 kg body weight in a 3-month period with the help of an energy and LCT-restricted, MCT- and carbohydrate-rich diet in combination with an exercise program. CK levels, C16, C18, and C18:1 levels of his acylcarnitine profile and his blood pressure decreased during the period of weight reduction. Patient 2 (male, 39 years) has a VLCAD deficiency. Dietary advice was energy and LCT restriction, MCT and carbohydrate-enriched food with raw cornstarch added during the night. Patient 2 lost almost 40 kg body weight to 87.6 kg (BMI 25.1) in 2 years. CK, insulin, TG, and ALAT blood levels decreased.

Successful Weight Loss in Two Adult Patients Diagnosed with Late-Onset Long-Chain Fatty Acid Oxidation Defect