PROP Nutrition Management Guidelines
First Edition
March 2017, v.1.2
Updated: September 2017
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A limited number of educational resources are currently available to support nutrition management of PROP. Development of a toolkit specific to these guidelines is planned. 

Learning Objectives for Patients/Families

Learning objectives for individuals with PROP and their caretakers include:

  • Understanding the deficiency of the propionyl-CoA carboxylase (PCC) enzyme and the resulting impact of propionic acid accumulation on metabolism of specific amino acids
  • Recognizing the need to monitor signs and symptoms of metabolic decompensation
  • Recognizing the need for frequent contact with appropriate healthcare providers (e.g. metabolic dietitian, nurse, physician)
  • Acquiring skills needed to implement the dietary prescription
  • Realizing the importance of strict adherence to the dietary prescription
  • Recognizing the role of medical food in the dietary management
Resources for patients

($ = indicates available for purchase)

Support and Advocacy Groups

  • Organic Acidemia Association: OAA is a non-profit volunteer run organization dedicated to support, education and advocacy for persons with PROP and other organic acidemias.  Offers online resources for families and professionals, newsletters, research study announcements, and an biennual educational symposium often in conjunction with the Fatty acid oxidation support group.  OAA has a board of directors and medical advisory board. Past newsletters are available and indexed by topic.

  • Propionic Acidemia Foundation: PAF is dedicated to finding improved treatments and a cure for Propionic Acidemia by funding research and providing information and support to families and medical professionals.  Offers online resources for persons with PROP, their families and health care professionals, newsletters, and research study announcements.  PAF has a board of directors and medical advisory board. Past newsletters are available and indexed by topic.

  • CLIMB (Children Living with Inherited Metabolic Disorders): the United Kingdom's provider of free metabolic disease information to young people, adults, families, professionals and other interested groups. Their mission is to provide Metabolic Disease specific information, advice and support to children, young people, adults, families and professionals in the United Kingdom and to provide information and support to families worldwide, to fund educational and primary research programs and to investigate treatments and medical services.

  • Social Media: Propionic acidemia on Facebook, Instagram, Twitter, etc 

What is Propionic Acidemia?

  • STAR-G Genetic Fact Sheets for Propionic Acidemia (updated July 11, 2016) in English and Spanish, concise, but accurate fact sheets that are in a downloadable format.


  • Genetic Home References: (PROP website reviewed July 2007 published Jan 24 2017) provides a brief overview of this disorder and genetic inheritance, with a comprehensive list of links to many other resources in English

  • A Guide for the Family of a Child with Propionic Acidemia (2009):  a guide in English or Spanish, providing a clear definition of the disorder and treatment, appropriate for the family with a newly diagnosed infant on nutrition therapy.  Available to health care professionals for families from Abbott Nutrition, a division of Abbott Laboratories, Inc., PO Box 1317, Columbus, OH 43216, 1-800-986-8755 FREE.
  • Parents’ Guide to PA (June 2005): a printer-friendly booklet on PROP in English, available as a PDF for downloading, prepared by the California Department of Health Services Newborn Screening Program.

Nutrition Information

 Low protein cooking and Food protein tracking

  • Apples to Zucchini: A Collection of Favorite Low Protein Recipes (2005) by Virginia Schuett and Dorothy Corry: This book includes more than 500 recipes for low-protein diets, with special emphasis on fresh fruits and vegetables and recipes suitable for the entire family. Very few recipes include special low-protein ingredients. The focus is on a diversity of healthy foods and easily obtainable ingredients. Protein content is listed for each recipe. $

  • Food list for Propionic Acidemia (2008) This booklet in English lists VAL, ILE, MET, THR, PRO, carbohydrate and calorie content of portions of common foods by weight and household measurements. Includes management tips for persons following the PROP diet. Available free of charge upon request from Abbott Nutrition, a division of Abbott Laboratories, Inc., PO Box 1317, Columbus, OH 43216, 1-800-986-8755 FREE.
  • Bowes and Church’s Food Values of Portions Commonly Used, Nineteenth Edition (2010):  This book lists more than 6,300 common foods with their protein, calorie and nutrient content, organized by food groups.  Order online or at your local bookseller $
  • Online smartphone Websites and Apps of Low protein recipes and to track Protein and Calorie content in foods.
Resources for providers

($ = indicates available for purchase)

Find a Nearby PROP Health Care Provider

  • GMDI:  Genetic Metabolic Dietitians International: Find a Metabolic Dietitian in Canada or the USA

PROP Medical Information Online

  • American College of Medical Genetics (ACMG) ACT SHEET and algorithm for PROP: information to guide providers through confirmatory testing following an abnormal newborn screen for PROP.  and


  • GeneReviews: a collection of expert-authored, peer-reviewed disease descriptions that apply genetic testing to the diagnosis, management, and genetic counseling of patients and families with specific inherited conditions.  Developed under the National Center for Biotechnology Information (NCBI), a division of the National Library of Medicine (NLM) at the National Institutes of Health (NIH).

  • Screening, Technology And Research in Genetics (STAR-G) Project: a multi-state collaborative effort, led by the Hawai`i Department of Health, to obtain research data, identify strategies and develop materials for addressing the financial, ethical, legal and social issues (FELSI) surrounding the use of tandem mass spectrometry (MS/MS) for neonatal metabolic screening of culturally and ethnically diverse populations, funded by the Health Resources and Services Administration (HRSA) through a Special Projects of Regional and National Significance (SPRANS) grant.  The STAR-G website includes general information on newborn screening, genetics and fact sheets on metabolic disorders, including PROP (last updated in 2005).

Clinical Nutrition Tools

  • Metabolic Pro: nutrient analysis software that includes amino acid data for foods and beverages, including medical foods and foods specially modified to be low in protein.  Developed with educational grants from Nutricia North America and BioMarin. Annual user license available from Genetic Metabolic Dietitians International (GMDI).  $