The Nutrition Management Guideline for individuals with propionic acidemia (PROP) is part of a larger project undertaken by the Southeast Regional Newborn Screening and Genetics Collaborative (SERC) (HRSA Region 3) and Genetic Metabolic Dietitians International (GMDI) to develop nutrition management guidelines for inherited metabolic disorders (IMD).

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

The target population includes individuals with PROP 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.

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 PROP and their families, advocates, and others.

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

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

• Describes the methodology used for development of evidence and consensus-based guidelines
• Provides background information about PROP, its diagnosis, biochemical pathway and nutrition treatment rationale
• Describes nutrition care in PROP, 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
• Suggests direction for future research