Efficacy of Phenylalanine- and Tyrosine-Restricted Diet in Alkaptonuria Patients on Nitisinone Treatment: Case Series and Review of Literature.

Pelin Teke Kisa, Semra Eroglu Erkmen, Hilal Bahceci,Zumrut Arslan Gulten,Ayca Aydogan, Ozge Kamer Karalar Pekuz,Tuba Yuce Inel,Taylan Ozturk,Sezer Uysal,Nur Arslan

Annals of nutrition & metabolism（2021）

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摘要

AKU patients should receive tyrosine/phenylalanine-restricted diet for reducing plasma tyrosine level to the safe range. Tyrosine/phenylalanine-free amino acid supplements can be safely used to enhance dietary compliance. Keratopathy and nutrient deficiency should be frequently monitored.

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关键词

Alkaptonuria,Amino acid supplementation,Nitisinone,Tyrosine/phenylalanine-restricted diet

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