Evaluation of a casein glycomacropeptide-based protein substitute, in the dietary management of NTBC-induced tyrosinaemia in patients with alkaptonuria: A prospective open-label study

IF 2.9 3区医学 Q3 NUTRITION & DIETETICS

Journal of Human Nutrition and Dietetics Pub Date : 2024-09-17 DOI:10.1111/jhn.13360

Shirley Judd, Ali Hutton, Grace Thomas, Heather Hill

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引用次数: 0

Abstract

Background

2-(2-Nitro-4-trifluoromethylbenzoyl)-1,3-cyclohexanedione (NTBC) treatment of alkaptonuria (AKU) leads to increased blood tyrosine levels, causing skin issues and potentially sight-threatening corneal keratopathy. Adherence to dietary management of NTBC-induced tyrosinemia, a low-protein diet with or without protein substitutes, can be difficult for patients. This 28-day interventional study evaluated a low tyrosine casein glycomacropeptide (cGMP) protein substitute (TYR sphere)®, a 20 g protein equivalent, cGMP-based protein substitute, in terms of adherence, palatability, usability, comparison to amino acid (AA)–based protein substitutes, gastrointestinal tolerance and metabolic control in adults with NTBC-induced tyrosinaemia.

Methods

Four adults (mean 61.1 years, range 53.3–69.3 years) with AKU and NTBC-induced tyrosinaemia were recruited from the United Kingdom National Alkaptonuria Centre (NAC). The cGMP protein substitute was prescribed based on individual nutritional requirements, replacing ≥1 AA-based protein substitute. Participants recorded product-related data in study diaries, using five-point Likert scales and daily and weekly logs. To determine metabolic control, prestudy blood tyrosine levels were compared to weekly blood spot tests during the study.

Results

Median cGMP protein substitute adherence was 98%. Most participants rated palatability and usability positively, and preferred cGMP protein substitute to AA-based products. There were no notable gastrointestinal changes, and metabolic control was maintained.

Conclusions

cGMP protein substitute is a palatable and well-tolerated option in the dietary management of AKU patients with NTBC-induced tyrosinaemia.

Abstract Image

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评估一种基于酪蛋白糖化酶肽的蛋白质替代品，用于碱蛋白尿患者 NTBC 引起的酪氨酸血症的饮食治疗：前瞻性开放标签研究

背景2-(2-硝基-4-三氟甲基苯甲酰基)-1,3-环己二酮（NTBC）治疗烷丙尿症（AKU）会导致血液中酪氨酸水平升高，引起皮肤问题和可能危及视力的角膜角膜病变。对患者来说，坚持使用低蛋白饮食（含或不含蛋白质替代品）来治疗 NTBC 引起的酪氨酸血症可能很困难。这项为期 28 天的干预性研究评估了低酪氨酸酪蛋白糖肽（cGMP）蛋白质替代品（TYR sphere）®（一种 20 克蛋白质当量、基于 cGMP 的蛋白质替代品）对 NTBC 引起的酪氨酸血症成人患者的依从性、适口性、可用性、与基于氨基酸（AA）的蛋白质替代品的比较、胃肠道耐受性和代谢控制。方法从英国国家钾尿症中心（NAC）招募了四名患有 AKU 和 NTBC 引起的酪氨酸血症的成人（平均 61.1 岁，53.3-69.3 岁）。cGMP 蛋白替代品根据个人营养需求处方，替代≥1 种 AA 蛋白替代品。参与者在研究日记中使用五点李克特量表以及每日和每周日志记录产品相关数据。为了确定代谢控制情况，将研究前的血液酪氨酸水平与研究期间每周的血样检测进行了比较。大多数参与者对其适口性和可用性给予了积极评价，并认为 cGMP 蛋白替代品比 AA 类产品更受欢迎。结论cGMP蛋白替代品是一种适口性好、耐受性好的选择，可用于AKU NTBC引起的酪氨酸血症患者的饮食管理。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Journal of Human Nutrition and Dietetics 医学-营养学

CiteScore

5.30

自引率

15.20%

发文量

133

审稿时长

6-12 weeks

期刊介绍： Journal of Human Nutrition and Dietetics is an international peer-reviewed journal publishing papers in applied nutrition and dietetics. Papers are therefore welcomed on: - Clinical nutrition and the practice of therapeutic dietetics - Clinical and professional guidelines - Public health nutrition and nutritional epidemiology - Dietary surveys and dietary assessment methodology - Health promotion and intervention studies and their effectiveness - Obesity, weight control and body composition - Research on psychological determinants of healthy and unhealthy eating behaviour. Focus can for example be on attitudes, brain correlates of food reward processing, social influences, impulsivity, cognitive control, cognitive processes, dieting, psychological treatments. - Appetite, Food intake and nutritional status - Nutrigenomics and molecular nutrition - The journal does not publish animal research The journal is published in an online-only format. No printed issue of this title will be produced but authors will still be able to order offprints of their own articles.