Tetrahydrobiopterin Responsiveness in a Series of 53 Cases of Phenylketonuria and Hyperphenylalaninemia in Iran

Tetrahydrobiopterin Responsiveness in a Series of 53 Cases of Phenylketonuria and Hyperphenylalaninemia in Iran Publisher

Setoodeh A¹ ; Yarali B² ; Rabbani A³ ; Khatami S⁴ ; Shams S⁵

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1. Division of Pediatric Endocrinology, Children's Medical Center, Tehran, Iran
2. Division of Pediatric Neurology, Children's Medical Center, Tehran, Iran
3. Growth and Development Center, Children's Medical Center, Tehran, Iran
4. Division of Biochemistry, Pasteur Institute, Tehran, Iran
5. Division of Pathology, Children's Medical Center, Tehran, Iran

Source: Molecular Genetics and Metabolism Reports Published:2015

Abstract

To determine the prevalence of 6R-Tetrahydrobiopterin (BH4) responsive phenylketonuria (PKU) in 53 cases of patients with various classification of hyperphenylalaninemia and PKU Excluding the BH4 deficient type referring to children's medical center in Iran (phenylalanine 360-2420 μmol/L), the single dose of 20 mg/kg (Kuvan®) and duration of 24 h was used. Results Among the 4 different categories of mild hyperphenylalaninemia requiring treatment, mild, moderate and classic PKU, the BH4 responders were 90%, 35.7%, 5.6% and 0% respectively after 24 h. Conclusion BH4 responsiveness is more prevalent in mild hyperphenylalaninemia and mild PKU patients in Iran. © 2015 The Authors. Published by Elsevier Inc.

Style	Citing Format
MLA	Setoodeh A, et al.. "Tetrahydrobiopterin Responsiveness in a Series of 53 Cases of Phenylketonuria and Hyperphenylalaninemia in Iran." Molecular Genetics and Metabolism Reports, vol. 2, no. , 2015, pp. 77-79.
APA	Setoodeh A, Yarali B, Rabbani A, Khatami S, Shams S (2015). Tetrahydrobiopterin Responsiveness in a Series of 53 Cases of Phenylketonuria and Hyperphenylalaninemia in Iran. Molecular Genetics and Metabolism Reports, 2(), 77-79.
Chicago	Setoodeh A, Yarali B, Rabbani A, Khatami S, Shams S. "Tetrahydrobiopterin Responsiveness in a Series of 53 Cases of Phenylketonuria and Hyperphenylalaninemia in Iran." Molecular Genetics and Metabolism Reports 2, no. (2015): 77-79.
Harvard	Setoodeh A et al. (2015) 'Tetrahydrobiopterin Responsiveness in a Series of 53 Cases of Phenylketonuria and Hyperphenylalaninemia in Iran', Molecular Genetics and Metabolism Reports, 2(), pp. 77-79.
Vancouver	Setoodeh A, Yarali B, Rabbani A, Khatami S, Shams S. Tetrahydrobiopterin Responsiveness in a Series of 53 Cases of Phenylketonuria and Hyperphenylalaninemia in Iran. Molecular Genetics and Metabolism Reports. 2015;2():77-79.
BibTex	@article{ author = {Setoodeh A and Yarali B and Rabbani A and Khatami S and Shams S}, title = {Tetrahydrobiopterin Responsiveness in a Series of 53 Cases of Phenylketonuria and Hyperphenylalaninemia in Iran}, journal = {Molecular Genetics and Metabolism Reports}, volume = {2}, number = {}, pages = {77-79}, year = {2015} }
RIS	TY - JOUR AU - Setoodeh A AU - Yarali B AU - Rabbani A AU - Khatami S AU - Shams S TI - Tetrahydrobiopterin Responsiveness in a Series of 53 Cases of Phenylketonuria and Hyperphenylalaninemia in Iran JO - Molecular Genetics and Metabolism Reports VL - 2 IS - SP - 77 EP - 79 PY - 2015 ER -

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