PKU Nutrition Management Guidelines
Second Edition
March 2022, v.2.5
Updated: June 2022
Feedback/Comments :: View Release Notes
March 2022, v.2.5
Updated: June 2022
Feedback/Comments :: View Release Notes
- Overview
- Methodology
- Background
- Biochemical Pathway and Nutrition Treatment Rationale
- Nutrition Assessment
- Comparative Standards
- Nutrition Problem Identification
- Nutrition Intervention
- Nutrition Recommendations
- 1. Nutrition intervention for appropriate nutrient intake
- 2. Nutrition intervention to achieve appropriate blood PHE concentration
- 3. Strategies for nutrition intervention
- 4. Monitoring nutrition intervention
- 5. Nutrition intervention with alternative or adjunctive therapies
- 6. Nutrition intervention before, during and after pregnancy
- 7. Pegvaliase Therapy
- Monitoring and Evaluation
- Resources
- Benefits and Harms of Implementing the Recommendations
- Barriers to Implementation
- Areas for Future Research
- List of Tables
-
Literature Evidence Summary Tables
- T.1 Laboratory and Clinical Findings in PKU
- T.2 Classification of PKU
- T.3 Recommended Intakes of PHE, TYR and Protein for PKU Treated with Dietary Therapy
- T.4 Nutrition Problem Identification for PKU
- T.5 Comparison of Recommendations for Dietary Protein Intake for Infants and Children under 4 Years of Age to the 2015 GMDI/SERC Guideline Recommendation
- T.6 Classification of Medical Foods for PKU
- T.7 Recommendations for Nutrient Intake and Sources in the Dietary Treatment of PKU
- T.8 Monitoring Nutritional Management of PKU
- T.9 Recommendations for Neurocognitive Testing in PKU
- T.10 Recommended Dosing Regimen for Pegvaliase
PKU Toolkit Tables- T.11 Recommended Intake of PHE, TYR, Protein and Energy for an Infant with PKU
- T.12 Composition of Breast Milk, Infant Formula, and Medical Food for an Infant with PKU
- T.13 Calculation of Formula Mixture Containing Breast Milk and Medical Food for an Infant with PKU
- T.14 Calculation of Formula Mixture Containing Infant Formula and Medical Food for an Infant with PKU
- T.15 Monitoring Nutritional Management for an Infant with PKU
- T.16 Calculation of Modified Formula Mixture Using Breast Milk for an Infant with PKU
- T.17 Current Daily Nutrient Intake for an Older Infant with PKU
- T.18 Composition of Infant Formula and Medical Food for an Older Infant with PKU
- T.19 Calculation of Daily Nutrient Intake Goals for an Older Infant with PKU
- T.20 Sample Menu Introducing Solid Food for an Older Infant with PKU
- T.21 Recommended Intake of PHE, TYR, Protein and Energy for a Child, Adolescent or Adult with PKU on Dietary Therapy
- T.22 DRI Ranges for Recommended Intake of Protein and Energy for Childhood through Adulthood by Gender and Age
- T.23 Composition of Medical Food for a Child with PKU
- T.24 Calculation of Nutrient Intake Goals for a Child with PKU
- T.25 Sample Menu for a Child with PKU Eating Lunch at School
- T.26 Monitoring Nutritional Management of PKU in a Young Child
- T.27 Composition of Medical Food for a Young Adult with PKU
- T.28 Calculation of Dietary Intake for the Young Adult with PKU
- T.29 Sample Menu for a Young Adult with PKU Transitioning to Self Care
- T.30 Monitoring Nutritional Management of PKU in the Older Child, Adolescent and Adult
- T.31 Sample menu for a Young Adult with PKU Transitioning to Self Care with a Simplified Diet
- T.32 Composition of Medical Food for a Child with PKU Before Taking Sapropterin
- T.33 Calculation of Daily Nutrient Intake Goals for a Child with PKU Before Taking Sapropterin
- T.34 Baseline Blood PHE and PHE Intake for a Child with PKU Before Taking Sapropterin
- T.35 Blood PHE and Dietary PHE Intake for a Child with PKU During a Trial of Sapropterin
- T.36 Calculation of Daily Nutrient Intake Goals for a Child with PKU While Taking Sapropterin
- T.37 Sample Menu Liberalized for a child with PKU While Taking Sapropterin
- T.38 Recommended Intakes of PHE, TYR, Protein and Energy in Pregnancy for a Woman with PKU
- T.39 Composition of Medical Food in Pregnancy for a Woman with PKU
- T.40 Calculation of Daily Nutrient Intake Goals in Early Pregnancy for a Woman with PKU
- T.41 Sample Menu for a Woman with PKU in Early Pregnancy
- T.42 Recommendations for Healthy Weight Gain During Pregnancy
- T.43 Monitoring Nutritional Management of PKU During Pregnancy and Lactation
- T.44 Sample Menu for a Woman with PKU in Late Pregnancy
- T.45 Changes in Blood PHE and TYR, Dietary Intake, and Dosage for an Adult with PKU and on Dietary Therapy During a Trial of Pegvaliase
- T.46 Calculation of Dietary Intake for the Young Adult with PKU During Maintenance and Diet Adjustments on Pegvaliase
- T.47 Monitoring Nutritional Management of PKU in the Adolescent or Adult on Pegvaliase Therapy and a Normal Diet
- T.48 Recommended Intake of Protein and Energy for Adolescence through Adulthood by Gender and Age
- T.49 Changes in Blood PHE and TYR, Dietary Intake, and Dosage for an Adult with PKU on an Unrestricted Diet During a Trial of Pegvaliase
- T.50 Calculation of Daily Nutrient Intake Goals for an Adult with PKU Before Response to Pegvaliase Therapy
- T.51 Recommended Intake of PHE, TYR, Protein and Energy for an Adolescent or Adult with PKU on Dietary Therapy
- References
- Contributors
- Appendix A: Recommendation Rating Definitions
- Appendix B: Terms
- Disclaimer
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Nutrition Intervention
Overview
The recommendations in this guideline summarize the evidence and consensus on best practices for individuals with PKU in order to:
- Meet nutrient needs for optimal growth, development and health maintenance
- Maintain blood PHE in the treatment range
- Provide interventions to maximize adherence to therapy
- Monitor adequacy of interventions
- Modify nutrition recommendations when adjunctive or alternate therapies are employed
- Provide appropriate nutritional intervention to allow a normal pregnancy and healthy offspring for a woman with PKU
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