{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,8]],"date-time":"2026-03-08T02:41:51Z","timestamp":1772937711532,"version":"3.50.1"},"reference-count":51,"publisher":"MDPI AG","issue":"19","license":[{"start":{"date-parts":[[2022,9,29]],"date-time":"2022-09-29T00:00:00Z","timestamp":1664409600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"BioMarin Pharmaceutical Inc.","award":["0000001285"],"award-info":[{"award-number":["0000001285"]}]},{"name":"BioMarin Pharmaceutical Inc.","award":["0000054906"],"award-info":[{"award-number":["0000054906"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Nutrients"],"abstract":"<jats:p>Choline is an essential nutrient for brain development and function that is attained through high-protein foods, which are limited in the phenylalanine-restricted diet of people with phenylketonuria (PKU). This study compared choline consumption among individuals with PKU to a reference sample from the National Health and Nutrition Examination Survey (NHANES), and identified treatment and diet-related factors that may modulate choline needs. Participants were individuals with PKU (n = 120, 4\u201361 years) managed with dietary therapy alone (n = 49), sapropterin dihydrochloride for \u22651 year (n = 38), or pegvaliase for \u22651 year with no medical food (n = 33). NHANES participants were not pregnant or nursing and came from the 2015\u20132018 cycles (n = 10,681, 4\u201370 years). Dietary intake data were used to estimate total usual intake distributions for choline, and mean probability of adequacy (MPA) was calculated as a summary index of nutrient adequacy for four methyl-donor\/co-factor nutrients that impact choline utilization (folate, vitamin B12, vitamin B6, and methionine). Only 10.8% (SE: 2.98) of the total PKU sample (14.7% [SE: 4.03] of children; 6.8% [SE: 2.89] of adults) achieved the adequate intake (AI) for choline, while 12.2% (SE:0.79) of the NHANES sample met the recommended level. Adults receiving pegvaliase were the most likely to exceed the AI for choline (14.82% [SE: 4.48]), while adults who were on diet therapy alone were the least likely (5.59% [SE: 2.95]). Without fortified medical foods, individuals on diet therapy and sapropterin would not be able to achieve the AI, and MPA for other methyl donor\/co-factor nutrients would be reduced. More frequent monitoring of choline intake and increased choline fortification of medical foods could benefit this population.<\/jats:p>","DOI":"10.3390\/nu14194056","type":"journal-article","created":{"date-parts":[[2022,9,29]],"date-time":"2022-09-29T23:09:29Z","timestamp":1664492969000},"page":"4056","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Characterization of Choline Nutriture among Adults and Children with Phenylketonuria"],"prefix":"10.3390","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4015-2247","authenticated-orcid":false,"given":"Meriah S.","family":"Schoen","sequence":"first","affiliation":[{"name":"Nutrition and Health Sciences, Laney Graduate School, Emory University, Atlanta, GA 30322, USA"},{"name":"Department of Human Genetics, Emory University, Atlanta, GA 30322, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Usha","family":"Ramakrishnan","sequence":"additional","affiliation":[{"name":"Nutrition and Health Sciences, Laney Graduate School, Emory University, Atlanta, GA 30322, USA"},{"name":"Hubert Department of Global Health, Rollins School of Public Health, Emory University, Atlanta, GA 30322, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jessica A.","family":"Alvarez","sequence":"additional","affiliation":[{"name":"Nutrition and Health Sciences, Laney Graduate School, Emory University, Atlanta, GA 30322, USA"},{"name":"Department of Medicine, Emory University, Atlanta, GA 30322, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Thomas R.","family":"Ziegler","sequence":"additional","affiliation":[{"name":"Nutrition and Health Sciences, Laney Graduate School, Emory University, Atlanta, GA 30322, USA"},{"name":"Department of Medicine, Emory University, Atlanta, GA 30322, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xiangqin","family":"Cui","sequence":"additional","affiliation":[{"name":"Biostatistics and Bioinformatics, Rollins School of Public Health, Emory University, Atlanta, GA 30322, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rani H.","family":"Singh","sequence":"additional","affiliation":[{"name":"Nutrition and Health Sciences, Laney Graduate School, Emory University, Atlanta, GA 30322, USA"},{"name":"Department of Human Genetics, Emory University, Atlanta, GA 30322, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2022,9,29]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1417","DOI":"10.1016\/S0140-6736(10)60961-0","article-title":"Phenylketonuria","volume":"376","author":"Blau","year":"2010","journal-title":"Lancet"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"290","DOI":"10.1542\/peds.20.2.290","article-title":"The variability in manifestations of untreated patients with phenylketonuria (phenylpyruvic aciduria)","volume":"20","author":"Paine","year":"1957","journal-title":"Pediatrics"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"72","DOI":"10.1016\/j.ymgme.2016.04.008","article-title":"Updated, web-based nutrition management guideline for PKU: An evidence and consensus based approach","volume":"118","author":"Singh","year":"2016","journal-title":"Mol. 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