Department of Pediatric Nutrition and Metabolism, Cerrahpasa Faculty of Medicine, Istanbul University-Cerrahpasa, 34098 Istanbul, Turkey.
Nutrition and Dietetics PhD Programme, Institute of Health Sciences, Acibadem Mehmet Ali Aydınlar University, 34752 Istanbul, Turkey.
Nutrients. 2023 Jul 11;15(14):3105. doi: 10.3390/nu15143105.
The main treatment for pyridoxine-nonresponsive cystathionine-β-synthase deficiency is a strict diet. Most centers prescribe low-protein diets based on gram-protein exchanges, and all protein sources are weighed. The purpose of this study is to investigate the effects of a more liberal methionine (Met)-based diet with relaxed consumption of fruits and vegetables on metabolic outcomes and dietary adherence. Ten patients previously on a low-protein diet based on a gram-protein exchange list were enrolled. The natural protein exchange lists were switched to a "Met portion exchange list". Foods containing less than 0.005 g methionine per 100 g of the food were accepted as exchange-free foods. The switch to Met portioning had no adverse effects on the control of plasma homocysteine levels in terms of metabolic outcomes. It resulted in a significant reduction in patients' daily betaine dose. All patients preferred to continue with this modality. In conclusion, methionine-portion-based medical nutrition therapy with relaxed consumption of fruits and vegetables seems to be a good and safe option to achieve good metabolic outcomes and high treatment adherence.
吡哆醇非反应性胱硫醚-β-合酶缺乏症的主要治疗方法是严格的饮食。大多数中心根据克蛋白交换处方低蛋白饮食，所有蛋白质来源都称重。本研究旨在探讨更宽松的基于蛋氨酸（Met）的饮食方案，对代谢结果和饮食依从性的影响。研究纳入了 10 名先前接受基于克蛋白交换列表的低蛋白饮食的患者。天然蛋白交换列表被切换到“Met 部分交换列表”。每 100 克食物中含有少于 0.005 克蛋氨酸的食物被视为无交换食物。就代谢结果而言，Met 部分交换对控制血浆同型半胱氨酸水平没有不良影响。它导致患者每日甜菜碱剂量显著减少。所有患者都愿意继续这种治疗模式。总之，水果和蔬菜摄入量不受限制的基于蛋氨酸部分的医学营养治疗似乎是一种很好且安全的选择，可以达到良好的代谢结果和高治疗依从性。
