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

TEKE KISA P., KÖSE E., Ören N., ARSLAN N.

JOURNAL OF BASIC AND CLINICAL HEALTH SCIENCES, cilt.1, sa.3, ss.79-81, 2017 (ESCI) identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 1 Sayı: 3
  • Basım Tarihi: 2017
  • Doi Numarası: 10.5152/jbachs.2017.240
  • Dergi Adı: JOURNAL OF BASIC AND CLINICAL HEALTH SCIENCES
  • Derginin Tarandığı İndeksler: Emerging Sources Citation Index (ESCI), TR DİZİN (ULAKBİM)
  • Sayfa Sayıları: ss.79-81
  • Anahtar Kelimeler: Phenylalanine, classical phenylketonuria, LNAA supplementation, PKU, DYSFUNCTION, SUPPLEMENTS
  • Ankara Üniversitesi Adresli: Hayır

Özet

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.