Impact of Fruit and Vegetables in PKU

NCT05249218 | Completed

• 1 other identifier: 18/BC/DTS/NO/215
• Study Type: interventional
• Target: 16
• Locations: 1 country
• Sites: 1

Brief Summary

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.

Conditions

Phenylketonurias

Keywords

Fruit and vegetables; Phenylalanine; Animal protein; Vegetable protein

Outcome Measures

Primary Outcomes (2)

• 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.

  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.

  8 weeks

Secondary Outcomes (1)

• Blood Phe levels three times a week with PKU taking fruits and vegetables containing 76-100mg of Phe/100g without measurement during 6 months.

  6 months

Study Arms (2)

Group starting intervention with vegetable protein

ACTIVE COMPARATOR

Phase A followed by Phases B, C, D, E.

Other: Phase B - Extra 1 Phe exchange from fruits/vegetables containing Phe from 76-100mg/100g; Other: Phase C - Extra 2 Phe exchanges from fruits/vegetables containing Phe from 76-100mg/100g; Other: Phase D - Extra 1 Phe exchange from milk or yoghurt; Other: Phase E - Extra 2 Phe exchanges from milk or yoghurt

Group starting intervention with animal protein

ACTIVE COMPARATOR

Phase A followed by Phases D, E, B, C.

Other: Phase B - Extra 1 Phe exchange from fruits/vegetables containing Phe from 76-100mg/100g; Other: Phase C - Extra 2 Phe exchanges from fruits/vegetables containing Phe from 76-100mg/100g; Other: Phase D - Extra 1 Phe exchange from milk or yoghurt; Other: Phase E - Extra 2 Phe exchanges from milk or yoghurt

Interventions

Phase B - Extra 1 Phe exchange from fruits/vegetables containing Phe from 76-100mg/100g OTHER

Usual low Phe diet + 60g fruits/vegetables containing Phe from 76-100mg/100g

Group starting intervention with animal protein; Group starting intervention with vegetable protein

Phase C - Extra 2 Phe exchanges from fruits/vegetables containing Phe from 76-100mg/100g OTHER

Usual low Phe diet + 120g fruits/vegetables containing Phe from 76-100mg/100g

Group starting intervention with animal protein; Group starting intervention with vegetable protein

Phase D - Extra 1 Phe exchange from milk or yoghurt OTHER

Usual low Phe diet but 1 x 50 mg extra Phe exchange from milk or yoghurt

Group starting intervention with animal protein; Group starting intervention with vegetable protein

Phase E - Extra 2 Phe exchanges from milk or yoghurt OTHER

Usual low Phe diet but 2 x 50 mg extra Phe exchange from milk or yoghurt eaten daily

Group starting intervention with animal protein; Group starting intervention with vegetable protein

Eligibility Criteria

• Age: 5 Years - 12 Years
• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17)

You may qualify if:

• PKU patients diagnosed by NBS and BH4 deficiencies;
• Early and continuously treated;
• Adherent to their prescribed PKU diet consisting of a protein-restricted diet and Phe-free amino acid based protein substitute;
• In good general health as evidenced by medical history;
• Able to provide written informed consent/assent (parent/child);
• Able to comply with the study protocol and take study product according to the opinion of the PI;
• Patients with good metabolic control (age 5-12y, 120-360μmol/L) with 3 out of 4 levels within range prior to consent (if 2 of 4 above target range but one close to the upper limit, PI to decide if patient can enter the study).

You may not qualify if:

• Concomitant diseases / disorders such as renal or gut disease / disorders and diabetes;
• Dislikes fruits and vegetables containing 76-100 mg of Phe/100g e.g.brocolli or cauliflower;
• Treated with BH4 (sapropterin);
• Presence of intercurrent infection;
• Patients with poor metabolic control (age 5-12y, \>360μmol/L).

Sponsors & Collaborators

• Alex Pinto: lead

Study Sites (1)

Birmingham Women's and Children's Hospital

Birmingham, West Midlands, B4 6NH, United Kingdom

Location

MeSH Terms

Conditions

Phenylketonurias

Interventions

Yogurt

Condition Hierarchy (Ancestors)

Brain Diseases, Metabolic, Inborn; Brain Diseases, Metabolic; Brain Diseases; Central Nervous System Diseases; Nervous System Diseases; Amino Acid Metabolism, Inborn Errors; Metabolism, Inborn Errors; Genetic Diseases, Inborn; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Metabolic Diseases; Nutritional and Metabolic Diseases

Intervention Hierarchy (Ancestors)

Cultured Milk Products; Milk; Beverages; Diet, Food, and Nutrition; Physiological Phenomena; Fermented Foods; Dairy Products; Food; Food and Beverages

Study Design

• Study Type: interventional
• Phase: not applicable
• Allocation: RANDOMIZED
• Masking: NONE
• Purpose: OTHER
• Intervention Model: CROSSOVER
• Sponsor Type: OTHER
• Responsible Party: SPONSOR INVESTIGATOR
• PI Title: Research Dietitian

Model Details: This is a prospective, randomised controlled study over 21 weeks in children to assess how Phe intake from fruits and vegetables (containing 76-100 mg Phe/100g) vs. animal sources will impact on blood Phe control. This will be followed by a 6 months extension study examining the 'unlimited' use of fruits and vegetables containing Phe 76-100 mg/100g on blood Phe control.

Study Record Dates

• First Submitted: June 10, 2021
• First Posted: February 21, 2022
• Study Start: July 20, 2019
• Primary Completion: June 30, 2021
• Study Completion: June 30, 2021
• Last Updated: February 21, 2022

Record last verified: 2022-02

Locations

GB (1)