000008244 001__ 8244 000008244 005__ 20240411114223.0 000008244 0247_ $$2DOI$$a10.6083/np1939959 000008244 037__ $$aIR 000008244 041__ $$aeng 000008244 245__ $$aLarge neutral amino acid supplementation improves clinical efficacy of usual care in adult PKU 000008244 260__ $$bOregon Health and Science University 000008244 269__ $$a2020 000008244 336__ $$aAbstract 000008244 520__ $$aPKU requires lifelong management, but the childhood PKU diet does not accommodate for post-adolescent patient variations, including: (1) the diet's effect on blood phenylalanine (Phe) concentrations, and (2) the therapeutic benefits of prescribed blood Phe targets. Most adult patients discontinue the inflexible diet, and are exposed to PKU's adverse effects. Research supports large neutral amino acids (LNAAs) as potential alternative strategy; we propose an additive LNAA strategy. We aim to evaluate the clinical efficacy of a combined treatment strategy: usual care (the PKU diet) with LNAA supplementation. 000008244 540__ $$fCC BY 000008244 542__ $$fIn copyright - joint owners 000008244 650__ $$aPhenylketonurias$$023968 000008244 650__ $$aNutrition Therapy$$035502 000008244 6531_ $$aorphan products 000008244 6531_ $$aamino acid supplementation 000008244 6531_ $$amedical foods 000008244 6531_ $$ainherited metabolic diseases 000008244 692__ $$aOregon Clinical and Translational Research Institute$$041494 000008244 7001_ $$aPrince, Annie$$uOregon Health and Science University$$041354 000008244 7001_ $$aDepenweiller, Maria$$uOregon Health and Science University$$041354 000008244 7001_ $$aChambers, Ash$$uOregon Health and Science University$$041354 000008244 7001_ $$aAhring, Kirsten$$uOregon Health and Science University$$041354 000008244 7001_ $$aMoseley, Kathryn$$uOregon Health and Science University$$041354 000008244 7001_ $$aKumru, Burcu$$uOregon Health and Science University$$041354 000008244 711__ $$aResearch Week$$uOregon Health and Science University$$d2020 000008244 8564_ $$90d7e8cb9-3d03-4791-aea0-50c0b05b5219$$s51380$$uhttps://digitalcollections.ohsu.edu/record/8244/files/Annie-Prince.pdf 000008244 905__ $$a/rest/prod/np/19/39/95/np1939959 000008244 980__ $$aResearch Week