Nutrition Management Guidelines for PKU | Southeast Regional Genetics Network

Management

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PKU Nutrition Management Guidelines

Second Edition
March 2022, v.2.5
Updated: June 2022
Feedback/Comments :: View Release Notes

Overview
- Nutrition Guidelines Project
- Purpose
- Target Population
- Intended Users of the Guidelines
- Sponsor Organization
- Funding
- Content Highlights
Methodology
- Overview
- Question Formulation
- Search Process
- Critical Appraisal and Abstraction
- Evidence Summary
- Development of Recommendations
- Portal Application
- Plan for Updating
Background
- Introduction to Guidelines
- Newborn Screening
- Confirmatory Testing
- Genetics
Biochemical Pathway and Nutrition Treatment Rationale
- Biochemical Pathway Figure
- Nutrition Treatment Rationale
Nutrition Assessment
- Patient History
- Anthropometrics
- Nutrition-focused Physical Findings
- Laboratory Findings, Medical Tests and Procedures
- Food and Nutrition-related History
Comparative Standards
- Overview
- Comparison of nutrient intake to established standards
Nutrition Problem Identification
- Overview
- Common Nutrition Diagnoses
Nutrition Intervention
- Overview
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
- Biomarkers to Monitor
Resources
- Overview
- Learning Objectives for Patients/Families
- Resources for patients
- Resources for providers
Benefits and Harms of Implementing the Recommendations
- Overview
- Potential Harms
- Potential Benefits
Barriers to Implementation
- Overview
- Infrastructure
- Patient-related
- Regulatory
Areas for Future Research
- Areas for Future Research
List of 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
- Analyzed Literature
- Reference Materials
Contributors
- Key Project Personnel
Appendix A: Recommendation Rating Definitions
- Strength of Recommendation Rating
- Clinical Action/Application
Appendix B: Terms
- Important Terms Used in This Document
Disclaimer
- Disclaimer

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