背景:关于苯丙酮尿症(PKU)患者天然蛋白耐受性较高的食物选择,缺乏实用指导。这对于引入可以改善蛋白质耐受性的辅助药物治疗特别重要。
目的:制定一套指南,将高蛋白食物引入PKU患者的饮食中,这些患者每天可耐受10g蛋白质。
方法:2022年1月,一份26项食物组问卷,列出一系列含有蛋白质的食物,从5到20克/100克,已发送给所有英国遗传代谢疾病组(BIMDG)营养师(n=80;26个遗传代谢疾病[IMD]中心)。他们被要求在他们的IMD饮食团队中考虑何时建议将26种含蛋白质的食物组中的每一种引入患者的饮食中,这些食物可以耐受每天10g至60g的蛋白质。选择获得IMD饮食团队多数票的每个食物组的患者蛋白质耐受性作为引入的耐受性阈值。2022年3月,使用德尔福方法举行了一次虚拟会议,讨论并达成最终共识。
结果:来自22/26个IMD中心的营养师(85%)(11名儿科,11名成人)。对于耐受蛋白质≥15克/天的患者,同意包含以下食物:无麸质面食,无麸质面粉,普通面包,奶酪涂抹,软奶酪,和小扁豆在盐水中;对于蛋白质耐受性≥20g/天:坚果,硬奶酪,普通面粉,肉/鱼,和基于植物的替代产品(含5-10克/100克蛋白质),普通意大利面,种子,鸡蛋,干豆类,添加酵母提取物涂抹片;对于蛋白质耐受性≥30g/天:添加肉类/鱼类和基于植物的替代产品(含有10-20g/100g蛋白质);对于蛋白质耐受性≥40g/天:添加肉类/鱼类和基于植物的替代品(含有20g/100g蛋白质)。
结论:来自22个英国中心的IMD营养师的这一英国共识首次描述了根据个体患者蛋白质耐受性对高蛋白质食物的适用性和分配。它为卫生专业人员提供了宝贵的指导,使他们能够规范实践并为患者提供合理的建议。
Introduction: There is little practical guidance about suitable food choices for higher natural protein tolerances in patients with phenylketonuria (PKU). This is particularly important to consider with the introduction of adjunct pharmaceutical treatments that may improve protein tolerance. Aim: To develop a set of
guidelines for the introduction of higher protein foods into the diets of patients with PKU who tolerate >10 g/day of protein. Methods: In January 2022, a 26-item food group questionnaire, listing a range of foods containing protein from 5 to >20 g/100 g, was sent to all British Inherited Metabolic Disease Group (BIMDG) dietitians (n = 80; 26 Inherited Metabolic Disease [IMD] centres). They were asked to consider within their IMD dietetic team when they would recommend introducing each of the 26 protein-containing food groups into a patient’s diet who tolerated >10 g to 60 g/day of protein. The patient protein tolerance for each food group that received the majority vote from IMD dietetic teams was chosen as its tolerance threshold for introduction. A virtual meeting was held using Delphi methodology in March 2022 to discuss and agree final
consensus. Results: Responses were received from dietitians from 22/26 IMD centres (85%) (11 paediatric, 11 adult). For patients tolerating protein ≥15 g/day, the following foods were agreed for inclusion: gluten-free pastas, gluten-free flours, regular bread, cheese spreads, soft cheese, and lentils in brine; for protein tolerance ≥20 g/day: nuts, hard cheeses, regular flours,
meat/fish, and plant-based alternative products (containing 5−10 g/100 g protein), regular pasta, seeds, eggs, dried legumes, and yeast extract spreads were added; for protein tolerance ≥30 g/day:
meat/fish and plant-based alternative products (containing >10−20 g/100 g protein) were added; and for protein tolerance ≥40 g/day: meat/fish and plant-based alternatives (containing >20 g/100 g protein) were added. Conclusion: This UK
consensus by IMD dietitians from 22 UK centres describes for the first time the suitability and allocation of higher protein foods according to individual patient protein tolerance. It provides valuable guidance for health professionals to enable them to standardize practice and give rational advice to patients.