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Ideal dose of protein substitute intake. What do protein substitues do ?. provide 21 amino acids help growth lower phenylalanine levels some provide vitamins and minerals. How much do we give?. School of Thought 1. UK advocate generous quantities of protein substitute.
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Ideal dose of protein substitute intake
What do protein substitues do? • provide 21 amino acids • help growth • lower phenylalanine levels • some provide vitamins and minerals
School of Thought 1 UK advocate generous quantities of protein substitute
School of Thought 2 children with PKU don’t need big doses of protein substitute
Aim To investigate if a lower dose of protein substitute can achieve the same or better control when compared to the dosage recommended by the UK
Who did we study? • 25 children • 2-10y (median 6y) • 13 girls; 12 boys • dietary treatment: - 5.5 x 50 mg phenylalanine exchanges - median protein equivalent intake: 2.2 g/kg/day
Dosages of protein substitute • High dose - 2 g/kg/day protein equivalent • Low dose - 1.2 g/kg/day protein equivalent
7 week study Week 1 Week 2 Week 3 Week 4 Week 5 Week 6 Week 7
Week 1 Control normal dose of protein substitute
Week 1 Control day 5 6 7 morning night
Week 2 and 3 High
Week 2 and 3 High Low
Week 3 day 5 6 7 1 2 3 4 Morning Night
Week 4 and 5 Control normal dose of protein substitute
Week 5 Control 5 6 7 morning night
Week 6 and 7 High Low
Week 7 day 5 6 7 1 2 3 4 Morning Night
Change in morning blood phenylalanine concentrations
Median change in blood phenylalanine concentrations a.m. p.m.
What are the reasons for differences in change in blood phe levels? • age: no correlation • exchanges: no correlation • PKU mutations • calorie intake
What are the reasons for differences in change in blood phe levels? • age: no correlation • exchanges: no correlation • PKU mutations: coming soon • calorie intake
Summary • lower dosages of protein substitute increased plasma phenylalanine concentrations • in some children, it led to very poor phe control • individual differences may be due to severity of PKU • calories contributed by protein substitute is important
Benefits UK • evidence to justify dosage of protein substitute given • important to justify prescription costs None UK countries • should help improve dietary control