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

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...

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Autores principales: Hansen, Joyanna, Hollander, Suzanne, Drilias, Nicoletta, Van Calcar, Sandra, Rohr, Fran, Bernstein, Laurie
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley & Sons, Inc. 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7203646/
https://www.ncbi.nlm.nih.gov/pubmed/32395413
http://dx.doi.org/10.1002/jmd2.12106
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author Hansen, Joyanna
Hollander, Suzanne
Drilias, Nicoletta
Van Calcar, Sandra
Rohr, Fran
Bernstein, Laurie
author_facet Hansen, Joyanna
Hollander, Suzanne
Drilias, Nicoletta
Van Calcar, Sandra
Rohr, Fran
Bernstein, Laurie
author_sort Hansen, Joyanna
collection PubMed
description 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.
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spelling pubmed-72036462020-05-11 Simplified Diet for nutrition management of phenylketonuria: A survey of U.S. metabolic dietitians Hansen, Joyanna Hollander, Suzanne Drilias, Nicoletta Van Calcar, Sandra Rohr, Fran Bernstein, Laurie JIMD Rep Research Reports 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. John Wiley & Sons, Inc. 2020-04-08 /pmc/articles/PMC7203646/ /pubmed/32395413 http://dx.doi.org/10.1002/jmd2.12106 Text en © 2020 The Authors. Journal of Inherited Metabolic Disease published by John Wiley & Sons Ltd on behalf of SSIEM. This is an open access article under the terms of the http://creativecommons.org/licenses/by/4.0/ License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.
spellingShingle Research Reports
Hansen, Joyanna
Hollander, Suzanne
Drilias, Nicoletta
Van Calcar, Sandra
Rohr, Fran
Bernstein, Laurie
Simplified Diet for nutrition management of phenylketonuria: A survey of U.S. metabolic dietitians
title Simplified Diet for nutrition management of phenylketonuria: A survey of U.S. metabolic dietitians
title_full Simplified Diet for nutrition management of phenylketonuria: A survey of U.S. metabolic dietitians
title_fullStr Simplified Diet for nutrition management of phenylketonuria: A survey of U.S. metabolic dietitians
title_full_unstemmed Simplified Diet for nutrition management of phenylketonuria: A survey of U.S. metabolic dietitians
title_short Simplified Diet for nutrition management of phenylketonuria: A survey of U.S. metabolic dietitians
title_sort simplified diet for nutrition management of phenylketonuria: a survey of u.s. metabolic dietitians
topic Research Reports
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7203646/
https://www.ncbi.nlm.nih.gov/pubmed/32395413
http://dx.doi.org/10.1002/jmd2.12106
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