PKU Nutrition Management Guidelines
First Edition
March 2015, v.1.12
Current version: v.2.5
Updated: August 2016
Feedback/Comments :: View Release Notes
March 2015, v.1.12
Current version: v.2.5
Updated: August 2016
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
- 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
- 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
PKU Toolkit Tables- T.10 Recommended Intake of PHE, TYR, Protein and Energy for an Infant with PKU
- T.11 Composition of Breast Milk, Infant Formula, and Medical Food for an Infant with PKU
- T.12 Calculation of Formula Mixture Containing Breast Milk and Medical Food for an Infant with PKU
- T.13 Calculation of Formula Mixture Containing Infant Formula and Medical Food for an Infant with PKU
- T.14 Monitoring Nutritional Management for an Infant with PKU
- T.15 Calculation of Modified Formula Mixture Using Breast Milk for an Infant with PKU
- T.16 Current Daily Nutrient Intake for an Older Infant with PKU
- T.17 Composition of Infant Formula and Medical Food for an Older Infant with PKU
- T.18 Calculation of Daily Nutrient Intake Goals for an Older Infant with PKU
- T.19 Sample Menu Introducing Solid Food for an Older Infant with PKU
- T.20 Recommended Intake of PHE, TYR, Protein and Energy for a Child, Adolescent or Adult with PKU
- T.21 DRI Ranges for Recommended Intake of Protein and Energy for Childhood through Adulthood by Gender and Age
- T.22 Composition of Medical Food for a Child with PKU
- T.23 Calculation of Nutrient Intake Goals for a Child with PKU
- T.24 Sample Menu for a Child with PKU Eating Lunch at School
- T.25 Monitoring Nutritional Management of PKU in a Young Child
- T.26 Composition of Medical Food for a Young Adult with PKU
- T.27 Calculation of Dietary Intake for the Young Adult with PKU
- T.28 Sample Menu for a Young Adult with PKU Transitioning to Self Care
- T.29 Monitoring Nutritional Management of PKU in the Older Child, Adolescent and Adult
- T.30 Sample menu for a Young Adult with PKU Transitioning to Self Care with a Simplified Diet
- T.31 Composition of Medical Food for a Child with PKU Before Taking Sapropterin
- T.32 Calculation of Daily Nutrient Intake Goals for a Child with PKU Before Taking Sapropterin
- T.33 Baseline Blood PHE and PHE Intake for a Child with PKU Before Taking Sapropterin
- T.34 Blood PHE and Dietary PHE Intake for a Child with PKU During a Trial of Sapropterin
- T.35 Calculation of Daily Nutrient Intake Goals for a Child with PKU While Taking Sapropterin
- T.36 Sample Menu Liberalized for a child with PKU While Taking Sapropterin
- T.37 Recommended Intakes of PHE, TYR, Protein and Energy in Pregnancy for a Woman with PKU
- T.38 Composition of Medical Food in Pregnancy for a Woman with PKU
- T.39 Calculation of Daily Nutrient Intake Goals in Early Pregnancy for a Woman with PKU
- T.40 Sample Menu for a Woman with PKU in Early Pregnancy
- T.41 Recommendations for Healthy Weight Gain During Pregnancy
- T.42 Monitoring Nutritional Management of PKU During Pregnancy and Lactation
- T.43 Sample Menu for a Woman with PKU in Late Pregnancy
- References
- Contributors
- Appendix A: Recommendation Rating Definitions
- Appendix B: Terms
- Disclaimer
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Resources
Overview
Educational resources and tools are currently available to support nutrition management of PKU. A toolkit containing additional resources specific to these guidelines is being developed.
Learning Objectives for Patients/Families
Learning objectives for individuals with PKU and their caretakers include:
- Understanding the basic metabolism of PHE and the role of PAH deficiency
- Recognizing the need to monitor blood PHE
- Recognizing the need for frequent contact with appropriate healthcare providers (e.g. metabolic dietitian, nurse, physician)
- Acquiring skills needed to implement the dietary prescription
- Realizing the importance of strict adherence to the dietary prescription
- Recognizing the role of medical food in the dietary management
Resources for patients
A compilation of resources for individuals with PKU and their families developed by National PKU Alliance is available at:
Resources for providers
A compilation of resources for metabolic dietitians developed by GMDI is available at:
www.gmdi.org (accessible only by GMDI members)
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SOUTHEAST REGIONAL GENETICS NETWORK —
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