A prospective, open label, acceptability study to evaluate PKU, MSUD, HCU, TYR and GA express plus in the dietary management of 40 patients with IEM. The following parameters will be assessed: adherence to prescribed dietary intakes, palatability, usability, gastrointestinal tolerance, clinically relevant routine biochemical parameters, timeframe to transition and contribution of the express plus range to overall protein substitute intake over a 28 day period.
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Questionnaire of self-reported adherence to the prescribed amount of study product
Timeframe: Days 1-28
Transition time
Timeframe: Transition period Weeks 1-6 (maximum)
Change in gastrointestinal tolerance from week 1 to week 4
Timeframe: Days 1-7 and days 21-28
Product acceptability rated on a Likert scale by the patient after 28-day intake
Timeframe: Visit 2 (end of 28 day evaluation period)
Change in general neophobia
Timeframe: Visit 1 (Day 1) and Visit 2 (end of 28 day evaluation period)
Change in food neophobia
Timeframe: Visit 1 (Day 1) and Visit 2 (end of 28 day evaluation period)
Nutritional suitability: change Phenylalanine levels
Timeframe: Visit 1 until visit 2 (day 28)
Nutritional suitability: change Tyrosine levels
Timeframe: Visit 1 until visit 2 (day 28)