Management
Guidelines
Portal
DNDF Model
PKU Nutrition Management Guidelines
First Edition
March 2015, v.1.12
Updated: August 2016
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Overview
Nutrition Guidelines Project

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).

Purpose

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.

Target Population

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.

Intended Users of the Guidelines

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.

Sponsor Organization

The development of nutrition guidelines is a joint effort between the Southeast Regional Newborn Screening Collaborative (SERC) and Genetic Metabolic Dietitians International (GMDI).

Funding

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.

Content Highlights

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
© 2006-2015   —   SOUTHEAST REGIONAL GENETICS NETWORK   —   A HRSA SUPPORTED PARTNERSHIP   —   GRANT #UH7MC30772