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Ideal dose Ideal dose of protein of protein substitute substitute intake intake

Ideal dose of protein substitute intake

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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. - PowerPoint PPT Presentation

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Page 1: Ideal dose  of protein  substitute      intake

Ideal doseIdeal dose of proteinof protein substitute substitute intake intake

Page 2: Ideal dose  of protein  substitute      intake

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

Page 3: Ideal dose  of protein  substitute      intake

How much do How much do we we give?give?

Page 4: Ideal dose  of protein  substitute      intake

School of Thought School of Thought 11

UK advocate generous quantities of protein substitute

Page 5: Ideal dose  of protein  substitute      intake

School of Thought School of Thought 22

children with PKU don’t need big doses of protein substitute

Page 6: Ideal dose  of protein  substitute      intake

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

Page 7: Ideal dose  of protein  substitute      intake

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

Page 8: Ideal dose  of protein  substitute      intake

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

Page 9: Ideal dose  of protein  substitute      intake

Week 1

Week 2

Week 3

Week 4

Week 5

Week 6

Week 7

7 week study

Page 10: Ideal dose  of protein  substitute      intake

Week 1

ControlControl

normal dose of protein substitute

Page 11: Ideal dose  of protein  substitute      intake

Week 1 ControlControl

5 6 7

morning

night

day

Page 12: Ideal dose  of protein  substitute      intake

Week 2 and 3

High

Page 13: Ideal dose  of protein  substitute      intake

Week 2 and 3

High Low

Page 14: Ideal dose  of protein  substitute      intake

Week 3

1 2 3 4 5 6 7day

Morning

Night

Page 15: Ideal dose  of protein  substitute      intake

Weighed food record

Page 16: Ideal dose  of protein  substitute      intake

Week 4 and 5

ControlControl

normal dose of protein substitute

Page 17: Ideal dose  of protein  substitute      intake

Week 5 ControlControl

5 6 7

morning

night

Page 18: Ideal dose  of protein  substitute      intake

Week 6 and 7

High Low

Page 19: Ideal dose  of protein  substitute      intake

Week 7

1 2 3 4 5 6 7day

Morning

Night

Page 20: Ideal dose  of protein  substitute      intake

Same food menu as week 3

Page 21: Ideal dose  of protein  substitute      intake

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

Page 25: Ideal dose  of protein  substitute      intake

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.

Page 26: Ideal dose  of protein  substitute      intake

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

Page 27: Ideal dose  of protein  substitute      intake

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

Page 28: Ideal dose  of protein  substitute      intake

Reduction in calorie intakeReduction in calorie intake

Page 29: Ideal dose  of protein  substitute      intake

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

Page 30: Ideal dose  of protein  substitute      intake

BenefitsBenefitsUKUKevidence to justify dosage of

protein substitute givenimportant to justify prescription

costsNone UK countriesshould help improve dietary

control