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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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Group starting intervention with vegetable protein | Active Comparator | Phase A followed by Phases B, C, D, E. |
|
| Group starting intervention with animal protein | Active Comparator | Phase A followed by Phases D, E, B, C. |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| 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 |
|
| Measure | Description | Time Frame |
|---|---|---|
| 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 |
| Measure | Description | Time Frame |
|---|---|---|
| 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 |
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Inclusion Criteria:
Exclusion Criteria:
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Birmingham Women's and Children's Hospital | Birmingham | West Midlands | B4 6NH | United Kingdom |
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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.
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| 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 |
|
| 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 |
|
| 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 |
|
| ID | Term |
|---|---|
| D010661 | Phenylketonurias |
| ID | Term |
|---|---|
| D020739 | Brain Diseases, Metabolic, Inborn |
| D001928 | Brain Diseases, Metabolic |
| D001927 | Brain Diseases |
| D002493 | Central Nervous System Diseases |
| D009422 | Nervous System Diseases |
| D000592 | Amino Acid Metabolism, Inborn Errors |
| D008661 | Metabolism, Inborn Errors |
| D030342 | Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D008659 | Metabolic Diseases |
| D009750 | Nutritional and Metabolic Diseases |
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| ID | Term |
|---|---|
| D015014 | Yogurt |
| ID | Term |
|---|---|
| D043302 | Cultured Milk Products |
| D008892 | Milk |
| D001628 | Beverages |
| D000066888 | Diet, Food, and Nutrition |
| D010829 | Physiological Phenomena |
| D000074421 | Fermented Foods |
| D003611 | Dairy Products |
| D005502 | Food |
| D019602 | Food and Beverages |
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