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Simplified Diet for nutrition management of phenylketonuria: A survey of U.S. metabolic dietitians.


ABSTRACT: Background:Phenylketonuria (PKU) is an inherited metabolic disorder affecting the conversion of phenylalanine (Phe) to tyrosine. Medical nutrition therapy, consisting of a Phe-restricted diet with medical formula, is the primary treatment for PKU. The Simplified Diet is an approach to PKU nutrition management that allows certain fruits, vegetables, and low-protein foods to be eaten without measuring or tracking, referred to as free/uncounted foods. There is no consensus on how to implement this approach in metabolic centers in the United States (U.S.), and clinical practice varies. Aim:This study describes the clinical experience of metabolic dietitians in U.S.-based metabolic centers related to the use and implementation of the Simplified Diet. Methods:A survey was developed and sent out to metabolic dietitians to query current clinical practices related to the Simplified Diet. Descriptive statistics were used to analyze responses. Results:Sixty-three dietitians managing ?5 patients with PKU in U.S.-based metabolic centers responded to the survey. Ninety-eight percent of survey respondents reported using some version of the Simplified Diet in clinical practice. The survey identified areas of strong agreement, including introduction of the Simplified Diet at 6 to 12?months of age. The survey also identified areas of widespread variability, including specific Phe or protein thresholds for free/uncounted foods, and whether or not to set daily quantity limits on these foods. Conclusions:Significant variability related to implementation of the Simplified Diet exists across U.S.-based metabolic centers. This practice variability may contribute to differences in the patient experience across centers and may indicate a need for development of clinical guidelines.

SUBMITTER: Hansen J 

PROVIDER: S-EPMC7203646 | BioStudies | 2020-01-01

REPOSITORIES: biostudies

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