The Effect of Large Neutral Amino Acids on Blood Phenylalanine Levels in Patients with Classical Phenylketonuria


Kisa P., Kose E., ÖREN N., ARSLAN N.

JOURNAL OF BASIC AND CLINICAL HEALTH SCIENCES, vol.1, no.3, pp.79-81, 2017 (ESCI) identifier

  • Publication Type: Article / Article
  • Volume: 1 Issue: 3
  • Publication Date: 2017
  • Doi Number: 10.5152/jbachs.2017.240
  • Journal Name: JOURNAL OF BASIC AND CLINICAL HEALTH SCIENCES
  • Journal Indexes: Emerging Sources Citation Index (ESCI), TR DİZİN (ULAKBİM)
  • Page Numbers: pp.79-81
  • Keywords: Phenylalanine, classical phenylketonuria, LNAA supplementation, PKU, DYSFUNCTION, SUPPLEMENTS
  • Dokuz Eylül University Affiliated: Yes

Abstract

Purpose: Phenylketonuria (PKU) is an inherited metabolic disease caused by low levels of the enzyme phenylalanine hydroxylase. Treatment includes dietary restriction of phenylalanine (Phe) and supplementation with tetrahydrobiopterin and large neutral amino acids (LNAAs). The purpose of this study is to evaluate the effect of LNAA therapy on blood Phe levels in patients undergoing treatment for at least 6 months.