intercurrent illness, stress, injury or surgery can trigger a catabolic event leading to increases in propionate metabolites and ammonia. Unless immediate intervention with reversal of catabolism are undertaken, patients with PROP run the risk of damage to their central nervous system that can be manifested in motor, intellectual and/or behavioral impairment. Outcome is therefore dependent on early diagnosis with initiation of treatment, appropriate treatment during catabolic events, and consistent treatment and monitoring to maintain appropriate plasma amino acids, ammonia, growth and health maintenance.
Inappropriate intake of medical food can impact growth, nutritional status and overall health. Excessive intake of foods with intact protein can, cause metabolic decompensation, and have an impact on the central nervous system. Over-restriction of propiogenic amino acids can impact growth and development. Pregnancy in a woman with PROP involves not only the typical PROP management, but also the increased needs of the pregnancy, the health of the fetus, the management of pregnancy-related illnesses and the rapid catabolic changes that occur after delivery. Liver transplantation may allow an individual with PROP to consume more intact protein, but without nutritional counseling during the transition, nutrient needs may not be met.
Lack of appropriate nutrition management can result in negative consequences. This can be the result of implementing only some of the recommendations, failing to monitor outcomes, and failing to adjust nutrient intake in response to growth or illness.
Implementing the recommendations would: