Paediatrics & Neonatology Unit, Guglielmo da Saliceto Hospital, Piacenza, Italy.
Rare and Metabolic Diseases Unit, AORN Santobono-Pausilipon, Naples, Italy.
J Int Med Res. 2022 Sep;50(9):3000605221125524. doi: 10.1177/03000605221125524.
To assess some quality of life (QOL) aspects of a low protein diet, using glycomacropeptide (GMP) as a protein substitute in patients with phenylketonuria (PKU).
This was a multicentre, prospective observational cohort, study. Metabolic control, nutritional parameters, and dietary adherence were assessed in patients with PKU before (T0), and six months after (T6) starting a low protein diet using GMP. Selected items from the PKU-QOL questionnaire were used to assess patients' acceptance of their modified diet.
18 patients from three Italian Centres, completed the study. With the exception of LDL-cholesterol and vitamin 25OH-D concentrations, there were no differences between T0 and T6 in metabolic or nutritional parameters. Data suggested that patients have a good acceptance of protein substitutes containing GMP, probably because of their improved palatability.
According to our patients' responses to items related to dietary regimen, GMP based protein substitutes do not appear to significantly affect QOL.
评估低蛋白饮食在一些生活质量（QOL）方面的情况，使用甘氨酰-脯氨酸二肽（GMP）作为苯丙酮尿症（PKU）患者的蛋白质替代物。
这是一项多中心、前瞻性观察队列研究。在开始使用 GMP 的低蛋白饮食之前（T0）和六个月后（T6），评估 PKU 患者的代谢控制、营养参数和饮食依从性。使用 PKU-QOL 问卷中的选定项目来评估患者对其改良饮食的接受程度。
来自意大利三个中心的 18 名患者完成了这项研究。除了 LDL 胆固醇和维生素 25OH-D 浓度外，代谢或营养参数在 T0 和 T6 之间没有差异。数据表明，患者对含有 GMP 的蛋白质替代品有较好的接受度，可能是因为它们改善了口感。
根据患者对与饮食方案相关项目的反应，基于 GMP 的蛋白质替代品似乎不会显著影响 QOL。
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