Propionic Acidemia Nutritional Guidelines


The Nutrition Guideline Committee is happy to announce that the Organic Acidemia Workgroup has published the “Propionic Acidemia (PROP) Nutrition Guidelines” in the February, 2019 issue of Molecular Genetics and Metabolism. The article is available and can be downloaded at no cost at

Publication of the PROP/PA Nutrition Guidelines in Molecular Genetics and Metabolism brings the latest evidence- and consensus-based nutrition management recommendations to the attention of clinicians, researchers, policy makers, insurers, and patients.
The new Nutrition Management Guidelines for PROP/PA provide:

  •  New directions including:
    • A greater emphasis on nutritional needs such as nutrient intake, nutritional interventions, supplementation, etc.
    • Less emphasis on  medical management which has been covered in previous publications;
    • Additional topics such as monitoring to ensure nutritional adequacy, nutritional issues with pregnancy and lactation, nutritional management for secondary complications such as pancreatitis, and finally a section addressing liver transplantation and the nutritional management before, during, and after the procedure.

Two consumer-oriented pieces, Frequently Asked Questions and a Consumer Summary, provide patients and families with information to use when interacting with their providers. The summary highlights key recommendations and suggests questions that patients and families may want to discuss with the metabolic team.

  • When patients and health care providers (HCPs) have the same information, they can work together as a team to identify the treatment that is best for the patient’s situation.
  • You can access these pieces at the Genetic Metabolic Dietitians International (GMDI) or Southeast Genetics Network websites located at  and  
  • The new recommendations were developed following a rigorous, and systematic process based on published standards for guideline development and modified to incorporate consensus methodology.  
  • This process took just over 2 years to complete and included:
    • 13 experienced metabolic dietitians who conducted an extensive review of 250 scientific and grey (unpublished) publications.
    • Two Delphi surveys of practice to obtain input from an additional 54 experienced HCPs,
    • A nominal group meeting, which included 7 HCPs and 2 parent group representatives, to clarify discrepancies and determine consensus regarding the proposed recommendations.
    • External review and field testing.
    • Inclusion of all reference materials and each step of the guideline development process stored on a secured web-based platform which are accessible on the websites listed above.       

We greatly appreciate the contributions and support of the clinicians and the patient community, who continue to help us work toward the goal of improving medical nutritional therapy for people living with propionic acidemia and their families and caregivers.

  • Next steps:
    • Nutrition Guideline Committee will continue to periodically update these guidelines as new evidence to support best clinical practices are discovered. 
    • In addition, a PROP tool kit is being developed to facilitate the implementation of the PROP Nutrition Guidelines, including educational resources. 

These nutritional guidelines are part of a multi-year project designed to optimize the nutritional management of individuals with inborn errors of metabolism. The guidelines establish nutritional recommendations based on the best available evidence, contribute to reduced uncertainty and variability of practice and identify needs for future research.  Nutritional management guidelines have already been published for other metabolic disorders including phenylketonuria (Singh R et al., Updated, web-based nutrition management guideline for PKU: an evidence and consensus based approach. (2016) Mol Genet Metab, 118(2); 72-83) and maple syrup urine disease (Frazier D et al., Nutrition management guideline for maple syrup urine disease: an evidence- and consensus-based approach. (2014) Mol Genet Metab, 112(3); 210-217), and in the very near future for fatty acid oxidation disorders. This project received funding from the Southeast Newborn Screening and Genetics Collaborative (by a Maternal and Child Health Bureau HRSA grant #2-U22 MC010979), and additional support from Genetic Metabolic Dietitians International (GMDI) to achieve the goal of developing nutritional guidelines for metabolic disorders where there is little published scientific evidence.  

Principle Investigators: Rani Singh, PhD, RD and Fran Rohr, MS, RD
Organic Acidemia Workgroup Co-Chairs: Elaina Jurecki, MS, RD and Keiko Ueda, MPH, RD
Organic Acidemia Workgroup members: Dianne Frazier PhD, MPH, RD, Christie Hussa, RD, MBA, Julie McClure, MPH, RD, Laura Nagy, MSc, RD, Lisa Obernolte, MS, RD, Matthew Rasberry, RD, LD, CNSC, Bridget Reineking, MS, RD, CD, Ann Marie Roberts, RD, CSP, CNSD, Amie Thompson, RD, LD, Steven Yannicelli, PhD, RD

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