Nutrients · 2025
Dietary Approaches in the Management of Adrenoleukodystrophy: Evidence Summary for Nutritional Tips.
СтатьяОбзорНазвание и аннотация: Перевод пока не подготовлен
Обработка статьи · этапы, ошибки и управление
Загрузка состояния…
Ошибки и ограничения
История · было → стало
Состояние обновляется каждые 5 секунд, пока блок открыт. Процент показывается только там, где известен объём работы. Пауза останавливает продолжение этапа; текущий запрос к источнику может завершиться.
Di Stefano Alice, Ricci Luca, Ferrari Davide, Frigerio Francesco, Minnetti Marianna, Fontana Mario, Donini Lorenzo M, Isidori Andrea M, Migliaccio Silvia, Poggiogalle Eleonora
Аннотация
Background: Adrenoleukodystrophy is a rare, inherited X-linked disease related to mutations in the ABCD1 gene. Peroxisomal β-oxidation is impaired, underpinning the tissue accumulation of very long-chain fatty acids (VLCFAs), especially in the central nervous system (i.e., the white matter and axons), adrenal glands, and testes. VLCFA accumulation contributes to oxidative stress, neuroinflammation, and progressive demyelination, leading to severe neurological sequelae. Though gene therapies and drug development are advancing, dietary management may still play a crucial role in modulating lipid metabolism and mitigating disease progression. Methods: A narrative review of studies published up to May 2025 in major scientific databases was conducted, focusing on biochemical and clinical outcomes, including VLCFA plasma modulation and nutritional status. Results: VLCFA restriction alone has shown limited efficacy due to the counteractive effect of endogenous synthesis. "Lorenzo's Oil" inhibits VLCFA elongation, yet with inconsistent clinical benefits. Novel dietary strategies, such as the "Bambino Diet" and innovative dietary supplements similar to Lorenzo's Oil, composed of glyceryl trioleate, glyceryl trierucate, and antioxidants, provide promising biochemical effects, such as reducing VLCFA plasma levels and improving lipid profiles. Malnutrition risk is also increased in X-ALD patients, underscoring the need for personalized nutritional interventions. Conclusions: Dietary strategies are one of the pillars of X-ALD management, to be further combined with pharmacological, gene therapies, and hematopoietic stem cell transplantation. Future research should refine emerging therapies, assess long-term effects, and develop personalized nutritional strategies.