In the UK, some fruits and vegetables are permitted without measurement in the diets of people with phenylketonuria (PKU). It is proven that fruits and vegetables containing phenylalanine (Phe) up to 75mg/100g (e.g. carrots, sweet potato, tomatoes) do not affect blood Phe control but it is unknown to what extent fruits and vegetables containing Phe from 76-100mg/100g (e.g. cauliflower, broccoli, beansprouts and asparagus) increase blood Phe levels. In addition, there is very little information about how differently animal and vegetable protein affect blood Phe levels. It is believed that plant protein may have less bioavailability and therefore have less impact on blood Phe control. The investigators aim is to perform a randomized controlled study over 21 weeks in children (5-12y) with PKU. Children will be studied during five different time periods, examining the effect on blood Phe control when increasing Phe intake from vegetables/fruits containing Phe from 76-100 mg/100g vs. animal sources. At the end of the study, fruits and vegetables containing 76- 100mg Phe/100g will continue to be given as "exchange-free" for 6 months. This is a practical, cost effective study and should bring benefit to all people with PKU following a very restrictive diet both within the UK and throughout the world.
Study Type
INTERVENTIONAL
Allocation
RANDOMIZED
Purpose
OTHER
Masking
NONE
Enrollment
16
Usual low Phe diet + 60g fruits/vegetables containing Phe from 76-100mg/100g
Usual low Phe diet + 120g fruits/vegetables containing Phe from 76-100mg/100g
Usual low Phe diet but 1 x 50 mg extra Phe exchange from milk or yoghurt
Usual low Phe diet but 2 x 50 mg extra Phe exchange from milk or yoghurt eaten daily
Birmingham Women's and Children's Hospital
Birmingham, West Midlands, United Kingdom
Blood Phe levels three times a week of patients with PKU taking extra 1 or 2 exchanges of fruits and vegetables containing 76-100mg of Phe/100g during 4 weeks each.
Time frame: 8 weeks
Blood Phe levels three times a week of patients with PKU taking extra 1 or 2 exchanges of animal protein during 4 weeks each.
Time frame: 8 weeks
Blood Phe levels three times a week with PKU taking fruits and vegetables containing 76-100mg of Phe/100g without measurement during 6 months.
Time frame: 6 months
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