Management
Guidelines
Portal
DNDF Model
MSUD Nutrition Management Guidelines
First Edition
February 2013, v.1.54
Updated: April 2018
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Overview
Nutrition Guidelines Project

The Nutrition Management Guideline for Individuals with Maple Syrup Urine Disease (MSUD) is part of a larger project undertaken by the Southeast Newborn Screening and Genetics Collaborative (SERC) (HRSA Region 3) and Genetic and Metabolic Dietitians International (GMDI) to develop nutrition management guidelines for inherited metabolic disorders (IMD).

Purpose

The purpose of this guideline is to foster optimum nutrition management of individuals with MSUD and reduce the uncertainty and variability in the management of this IMD.

Target Population

The target population includes individuals with MSUD at all life stages from the acute newborn period, through childhood and adolescence, to ongoing management in the adult years and during pregnancy. These guidelines include management during periods of health and illness.

Intended Users of the Guidelines

The guidelines are intended for use by metabolic dietitians, physicians, others of the health care team and researchers who collaborate to provide care and advice for individuals with MSUD and their families, advocates, and others.

Sponsor Organization

Sponsorship is shared by the Southeast Regional Newborn Screening and Genetics Collaborative (SERC) and Genetic Metabolic Dietitians International (GMDI).

Funding

Funding for this project has been provided by Health Resources and Services Administration (HRSA) grant number: #H46MC24090.

Content Highlights
  • Describes the methodology used for development of evidence and consensus-based guidelines
  • Provides background about MSUD, its diagnosis, biochemical pathway and nutrition treatment rationale
  • Describes nutrition care in MSUD, including assessment, nutrition problem identification, nutrition intervention with evidence- and consensus-based recommendations, and monitoring and evaluation
  • Lists specific recommendations with grades
  • Explores implementation considerations and needed research
© 2006-2015   —   SOUTHEAST REGIONAL GENETICS NETWORK   —   A HRSA SUPPORTED PARTNERSHIP   —   GRANT #UH7MC30772