Research and Innovation, Nutricia Ltd., White Horse Business Park, Trowbridge BA14 0XQ, UK.
Dietetic Department, Birmingham Children's Hospital, Birmingham B4 6NH, UK.
Nutrients. 2023 Aug 14;15(16):3580. doi: 10.3390/nu15163580.
(1) Background: Good adherence to a Phe-restricted diet supplemented with an adequate amount of a protein substitute (PS) is important for good clinical outcomes in PKU. Glycomacropeptide (cGMP)-PSs are innovative, palatable alternatives to amino acid-based PSs (AA-PS). This study aimed to evaluate a new cGMP-PS in liquid and powder formats in PKU. (2) Methods: Children and adults with PKU recruited from eight centres were prescribed at least one serving/day of cGMP-PS for 7-28 days. Adherence, acceptability, and gastrointestinal tolerance were recorded at baseline and the end of the intervention. The blood Phe levels reported as part of routine care during the intervention were recorded. (3) Results: In total, 23 patients (powder group, = 13; liquid group, = 10) completed the study. The majority assessed the products to be palatable (77% of powder group; 100% of liquid group) and well tolerated; the adherence to the product prescription was good. A total of 14 patients provided blood Phe results during the intervention, which were within the target therapeutic range for most patients ( = 11) at baseline and during the intervention. (4) Conclusions: These new cGMP-PSs were well accepted and tolerated, and their use did not adversely affect blood Phe control.
（1）背景：苯丙氨酸限制饮食辅以足够量的蛋白质替代物（PS）对于 PKU 的良好临床结局非常重要。糖巨肽（cGMP）-PS 是一种创新的、美味的替代基于氨基酸的 PS（AA-PS）。本研究旨在评估 PKU 中新型 cGMP-PS 的液体和粉末形式。
（2）方法：从八个中心招募的 PKU 儿童和成人每天至少服用一份 cGMP-PS 持续 7-28 天。在基线和干预结束时记录依从性、可接受性和胃肠道耐受性。在干预期间作为常规护理的一部分报告的血液苯丙氨酸水平也被记录。
（3）结果：共有 23 名患者（粉末组， = 13；液体组， = 10）完成了研究。大多数患者认为产品可口（粉末组 77%；液体组 100%）且耐受性良好；对产品处方的依从性良好。共有 14 名患者在干预期间提供了血液苯丙氨酸结果，这些结果在大多数患者（ = 11）的基线和干预期间均处于目标治疗范围内。
（4）结论：这些新型 cGMP-PS 被很好地接受和耐受，它们的使用不会对血液苯丙氨酸控制产生不利影响。
