March 2015, v.1.10
Current version: v.2.5
Updated: April 2015
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- 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
- References
- Contributors
- Appendix A: Recommendation Rating Definitions
- Appendix B: Terms
- Disclaimer
The PKU Nutrition Management Guideline is part of a larger project undertaken by the Southeast Newborn Screening and Genetics Collaboration – HRSA Region 3 (SERC) and the Genetic Metabolic Dietitians International (GMDI) to develop nutrition management guidelines for inherited metabolic disorders (IMD).
The purpose of this guideline is to foster optimal nutrition management of individuals with PKU and reduce the uncertainty and variability in the management of this disorder to allow for best outcomes.
The target population includes individuals with PKU at all life stages from the newborn period, through childhood and adolescence, to ongoing management in adult years, and during pregnancy. It also includes guidance for individuals who were late-diagnosed or are returning to treatment and those pairing dietary treatment with other treatment modalities.
The guideline is intended for use by all who provide care and advice for individuals with PKU, including: metabolic dietitians, physicians, and other health-care professionals, researchers, industry partners, public health personnel, third party health care payers, and PKU advocacy groups.
The development of nutrition guidelines is a joint effort between the Southeast Regional Newborn Screening Collaborative (SERC) and Genetic Metabolic Dietitians International (GMDI).
Funding for this project has been provided to SERC by the Health Resources and Services Administration (HRSA): grant # H46MC24090. GMDI received an unrestricted education grant from National PKU Alliance for partial funding of a dietitian consultant and the Nominal Group meeting.
Contents of the guideline include:
- Methodology used for development of the evidence- and consensus-based guideline
- Background about PKU: diagnosis, biochemical pathway, nutrition treatment rationale
- Description of nutrition care in management of PKU including: assessment, nutrition problem identification, nutrition intervention using evidence- and consensus-based recommendations, and monitoring and evaluation of outcome
- Rating of all recommendations based on the strength of the evidence
- Suggested implementation of the guideline recommendations
- Areas needing further research through collaborative studies