Ideal dose Ideal dose of protein of protein substitute intake substitute intake.
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Transcript of Ideal dose Ideal dose of protein of protein substitute intake substitute intake.
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Ideal doseIdeal dose of proteinof protein substitute substitute intake intake
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What do protein What do protein substitues dosubstitues do?
provide 21 amino provide 21 amino acidsacids
help growth help growth
lower phenylalanine lower phenylalanine levelslevels
some provide some provide vitamins and mineralsvitamins and minerals
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How much do How much do we we give?give?
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School of Thought School of Thought 11
UK advocate generous quantities of protein substitute
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School of Thought School of Thought 22
children with PKU don’t need big doses of protein substitute
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AimAimTo investigate if a To investigate if a lower dose of lower dose of protein substitute protein substitute can achieve can achieve the same or the same or better better control when control when compared to the compared to the dosage recommended dosage recommended by the UK by the UK
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Who did we study?
25 children25 children
2-10y (median 6y)2-10y (median 6y)
13 girls; 12 boys13 girls; 12 boys
dietary treatment:dietary treatment:
- 5.5 x 50 mg phenylalanine exchanges- 5.5 x 50 mg phenylalanine exchanges
- median protein equivalent intake: 2.2 - median protein equivalent intake: 2.2 g/kg/day g/kg/day
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Dosages of protein Dosages of protein substitutesubstitute
High doseHigh dose
- 2 g/kg/day - 2 g/kg/day protein protein
equivalent equivalent
Low doseLow dose
- 1.2 g/kg/day protein equivalent - 1.2 g/kg/day protein equivalent
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Week 1
Week 2
Week 3
Week 4
Week 5
Week 6
Week 7
7 week study
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Week 1
ControlControl
normal dose of protein substitute
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Week 1 ControlControl
5 6 7
morning
night
day
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Week 2 and 3
High
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Week 2 and 3
High Low
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Week 3
1 2 3 4 5 6 7day
Morning
Night
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Weighed food record
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Week 4 and 5
ControlControl
normal dose of protein substitute
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Week 5 ControlControl
5 6 7
morning
night
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Week 6 and 7
High Low
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Week 7
1 2 3 4 5 6 7day
Morning
Night
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Same food menu as week 3
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Subject 1Subject 1 girl 5y girl 5y 5½exchanges 5½exchanges
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Subject 2Subject 2 girl 6ygirl 6y 6 exchanges6 exchanges
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Subject 3Subject 3 girl 7 ygirl 7 y 7 exchanges7 exchanges
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Change in morning bloodChange in morning bloodphenylalanine concentrationsphenylalanine concentrations
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T IME O F D AY
p.m.a.m
CH
AN
GE
IN P
HE
NY
LALA
NIN
E F
RO
M C
ON
TR
OL
1000
800
600
400
200
0
-200
-400
LOW DOSE
HIGH DOSE
p < 0.001 n .s .p < 0.001 n .s .
Median change in blood phenylalanine concentrations
a.m. p.m.
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What are the reasons for What are the reasons for differences in change in differences in change in
blood phe levels?blood phe levels?
age: age: no correlationno correlationexchanges: exchanges: no correlationno correlationPKU mutations PKU mutations calorie intakecalorie intake
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What are the reasons for What are the reasons for differences in change in differences in change in
blood phe levels?blood phe levels?
age: age: no correlationno correlationexchanges: exchanges: no correlationno correlationPKU mutations: PKU mutations: coming coming
soonsooncalorie intakecalorie intake
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Reduction in calorie intakeReduction in calorie intake
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SummarySummarylower dosages of protein substitute increased lower dosages of protein substitute increased
plasma phenylalanine concentrationsplasma phenylalanine concentrations
in some children, it led to very poor phe in some children, it led to very poor phe controlcontrol
individual differences may be due to severity individual differences may be due to severity of PKUof PKU
calories contributed by protein substitute is calories contributed by protein substitute is important important
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BenefitsBenefitsUKUKevidence to justify dosage of
protein substitute givenimportant to justify prescription
costsNone UK countriesshould help improve dietary
control