Nutrition Management Guidelines for PKU | Southeast Regional Genetics Network

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PKU Nutrition Management Guidelines

First Edition
March 2015, v.1.12
Current version: v.2.5
Updated: August 2016
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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
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
- 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
- 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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This version is not current. Click to view the most recent edition (v.2.5, August 2016).

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 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 alternate or adjunctive therapies are employed
Provide appropriate nutritional intervention to allow a normal pregnancy and healthy offspring for a woman with PKU

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