Blood phenylalanine fluctuation in phenylketonuric children treated by BH4 or low-phenylalanine diet from birth
Abstract The prognosis of phenylketonuria (PKU) is related to the quality of metabolic control all life-long. PKU treatment is based on a low-Phe diet, 6R-tetrahydrobiopterin (BH4) treatment for the BH4-responsive PKU patients or enzyme replacement therapy. Fluctuations in blood phenylalanine (Phe)...
Main Authors: | , , , , , , |
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Format: | Article |
Language: | English |
Published: |
Nature Portfolio
2023-06-01
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Series: | Scientific Reports |
Online Access: | https://doi.org/10.1038/s41598-023-36550-1 |