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.
Autor: | Judd S; National Alkaptonuria Centre, Liverpool, UK., Hutton A; Vitaflo International Ltd, Liverpool, UK., Thomas G; Vitaflo International Ltd, Liverpool, UK., Hill H; Vitaflo International Ltd, Liverpool, UK. |
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Jazyk: | angličtina |
Zdroj: | Journal of human nutrition and dietetics : the official journal of the British Dietetic Association [J Hum Nutr Diet] 2024 Sep 17. Date of Electronic Publication: 2024 Sep 17. |
DOI: | 10.1111/jhn.13360 |
Abstrakt: | 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. (© 2024 Vitaflo (International) Limited. Journal of Human Nutrition and Dietetics published by John Wiley & Sons Ltd on behalf of British Dietetic Association.) |
Databáze: | MEDLINE |
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