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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
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
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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Feedback & Comments
This version is not current.
Click to view
the most recent edition (v.2.5, August 2016).
Appendix B: Terms
Important Terms Used in This Document
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