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Fenilketonüride Beslenme ve Yeni Tedavi Yaklaşımları

Yıl 2018, Cilt: 16 Sayı: 2, 187 - 198, 01.08.2018

İzzet Ülker Nevin Şanlıer

Öz

Fenilketonüri (PKU), fenilalanin hidrosilaz (PAH) geninde mutasyon sebebiyle gelişen otozomal resesif geçişli bir hastalıktır. Doğumdan sonra kan fenilalanin (FA) seviyesi normal aralıktayken, besin alımına başladıktan sonra bebeklerin kan FA seviyeleri yükselir. Hastalığa özgü tıbbi ve beslenme tedavisi yapılmadığında mental gerilik (IQ<50), deri-saç pigmentasyon bozukluları, büyüme geriliği, mikrosefali, epilepsi, davranış bozuklukları, hiperaktivite ve anksiyete gibi birçok klinik bulgu görülebilmektedir. Hastalığın tedavisinde çeşitli yöntemler bulunmakla birlikte beslenmenin önemi büyüktür. Beslenme tedavisinin amacı kan ve beyinde fenilalaninin birikmesini önlemektir. Ayrıca fenilalaninin tirozine dönüşümünün olmaması sebebiyle eksikliği gelişen tirozini yerine koymak ikincil amaçtır.
Beslenme tedavisiyle çocuğun büyüme ve gelişmesi normal seyreder ve komplikasyonlar engellenir. Ancak beslenme tedavisinin uygulanması küçük yaşlarda çok kolayken hastanın yaşı ilerledikçe diyete uyum güçleşmektedir. Yeni tedavi yaklaşımları ile diyete uyum kolaylaştırılabilmekte ve klinik bulgular engellenebilmektedir. Yeni tedavi yaklaşımlarından bazıları glikomakropeptid, büyük nötral aminoasitler ve tetrahidrobiyopterindir. Tetrahidrobiyopterin sadece tedaviye cevap veren klasik fenilketonürili hastalarda (önceden yapılan testlerle uygun olacağı öngörülen, seçilmiş hastalarda) ya da BH4 metabolizması bozukluğu olanlarda kullanılabilmektedir. Glikomakropeptid ve büyük nötral aminoasitler neredeyse tüm hastalarda kullanılabilmektedir.
Henüz çalışma aşamasında olan fenilalanin amonyak liyaz enziminin kullanılması, biriken fenilalaninin zararsız metabolitlere dönüşümünü sağlamaktadır. Ancak etkin bir beslenme tedavisinin uygulanması, sağlık ekibiyle aile arasında iletişim sağlanması ve aileye tedavi yönteminin anlatılmasıyla mümkün olur. Ayrıca beslenme tedavisinin yeni tedavi yaklaşımları ile desteklenmesi hastalığın bulgularının engellenmesi ve hastanın yaşam kalitesinin artırılmasında önemlidir.

Anahtar Kelimeler

Fenilketonüri Beslenme Yeni Tedavi Yaklaşımları

Kaynakça

  • 1. Blau N, van Spronsen FJ, Levy HL. Phenylketonuria. Lancet 2010; 376(9750): 1417-27.
  • 2. Sarkissian CN, Gamez A, Scriver CR. What we know that could influence future treatment of phenylketonuria. J Inherit Metab Dis 2009; 32(1):3-9.
  • 3. Ozturk Y. Metabolik Hastaliklarda Beslenme. http://wwwyesimozturkcom/PDF/YesimOzturk_MetabolikHastaliklardaBeslenme 2008pdf erişim tarihi 26/02/2016.
  • 4. Köksal G. Gökmen Özel H. Metabolik hastalıklarda beslenme. Ankara; Klasmat Matbaası; 2008, p.10-11.
  • 5. Ahring K, Bélanger-Quintana A, Dokoupil K, Ozel HG, Lammardo AM, MacDonald A, et al. Dietary management practices in phenylketonuria across European centres. Clin Nutr 2009; 28(3): 231-6. doi: http://dx.doi.org/10.1016/j.clnu.2009.03.004.
  • 6. Özer I. Fenilketonüri örneğinde doğumsal metabolik hastalıklarda genel tedavi yaklaşımı. Klin Pediatr. 2004; 3: 26-30(1).
  • 7. Singh RH, Rohr F, Frazier D, Cunningham A, Mofidi S, Ogata B, et al. Recommendations for the nutrition management of phenylalanine hydroxylase deficiency. Genetics in medicine : official J Am Col Med Genet 2014; 16(2): 121-31. doi: 10.1038/gim.2013.179. PubMed PMID: 24385075; PubMed Central PMCID: PMC3918542.
  • 8. Vockley J, Andersson HC, Antshel KM, Braverman NE, Burton BK, Frazier DM, et al. Phenylalanine hydroxylase deficiency: diagnosis and management guideline. Genetics in medicine : official journal of the American College of Medical Genetics. 2014;16(2):188-200. Epub 2014/01/05. doi: 10.1038/gim.2013.157. PubMed PMID: 24385074.
  • 9. Cunningham A, Bausell H, Brown M, Chapman M, DeFouw K, Ernst S, et al. Recommendations for the use of sapropterin in phenylketonuria. Mol Genet Metab 2012; 106(3): 269-76. doi: 10.1016/j.ymgme.2012.04.004. PubMed PMID: WOS:000305818600003.
  • 10. National Institutes of Health Consensus Development P. National Institutes of Health Consensus Development Conference Statement: phenylketonuria: screening and management, October 16-18, 2000. Pediatrics. 2001; 108(4): 972-82. PubMed PMID: 11581453.
  • 11. Ney DM, Blank RD, Hansen KE. Advances in the nutritional and pharmacological management of phenylketonuria. Curr Op Clin Nutr Metab Care 2014; 17(1): 61-8. doi: 10.1097/MCO.0000000000000002. PubMed PMID: 24136088; PubMed Central PMCID: PMC4004170.
  • 12. Hvas AM, Nexo E, Nielsen JB. Vitamin B12 and vitamin B6 supplementation is needed among adults with phenylketonuria (PKU). J Inherit Metab Dis 2006; 29(1): 47-53. Epub 2006/04/08. doi: 10.1007/s10545-006-0108-3. PubMed PMID: 16601867.
  • 13. Moseley K, Koch R, Moser AB. Lipid status and long-chain polyunsaturated fatty acid concentrations in adults and adolescents with phenylketonuria on phenylalanine-restricted diet. J Inherit Metab Dis 2002;25(1):56-64.
  • 14. Üstüner Top F. The difficulties of families of children with phenylketonuria: A qualitative study. HEAD 2015; 12(1): 62-8. doi: doi:10.5222/HEAD.2015.062.
  • 15. Blau N BP. Disorders of phenylalanine and tetrahydrobiopterin metabolism. Hoffmann Jl CJ, In: Physician's Guide to the Diagnosis, Treatment, and Follow-Up of Inherited Metabolic Diseases, Berlin Heidelberg 2006, p. 26-34.
  • 16. MacDonald A, Gokmen-Ozel H, van Rijn M, Burgard P. The reality of dietary compliance in the management of phenylketonuria. J Inherit Metab Dis 2010; 33(6): 665-70. Epub 2010/04/08. doi: 10.1007/s10545-010-9073-y. PubMed PMID: 20373144.
  • 17. Fuhrman MP, Galvin TA, Ireton-Jones CS, Thorpe J. Practice paper of the American Dietetic Association: Home care--opportunities for food and nutrition professionals. J Am Dietet Ass 2009; 109(6): 1092-100. Epub 2009/06/02. PubMed PMID: 19480085.
  • 18. Bakırel NA. Okul öncesi çağı klasik fenilketonürili çocukların malnütrisyon durumunun saptanması (Yüksek Lisans Tezi). Ankara: Gazi Üniversitesi; 2008.
  • 19. Etzel MR. Manufacture and use of dairy protein fractions. J Nutr 2004; 134(4): 996s-1002s. PubMed PMID: WOS:000220681700047.
  • 20. Bruck WM, Redgrave M, Tuohy KM, Lonnerdal B, Graverholt G, Hernell O, et al. Effects of bovine alpha-lactalbumin and casein glycomacropeptide-enriched infant formulae on faecal microbiota in healthy term infants. J Pediatr Gastr Nutr 2006; 43(5): 673-9. PubMed PMID: WOS:000241906800022.
  • 21. van Calcar SC, Ney DM. Food products made with glycomacropeptide, a low-phenylalanine whey protein, provide a new alternative to amino acid-based medical ffoods for nutrition management ofphenylketonuria. J Acad Nutr Diet 2012; 112(8): 1201-10. doi: 10.1016/j.jand.2012.05.004. PubMed PMID: WOS:000307318100011.
  • 22. Dangin M, Boirie Y, Garcia-Rodenas C, Gachon P, Fauquant J, Callier P, et al. The digestion rate of protein is an independent regulating factor of postprandial protein retention. Am J Physiol-Endoc M 2001; 280(2): E340-E8. PubMed PMID: WOS:000166453400020.
  • 23. Calbet JAL, MacLean DA. Plasma glucagon and insulin responses depend on the rate of appearance of amino acids after ingestion of different protein solutions in humans. J Nutr 2002; 132(8): 2174-82. PubMed PMID: WOS:000177398400010.
  • 24. Matalon R, Michals-Matalon K, Bhatia G, Grechanina E, Novikov P, McDonald JD, et al. Large neutral amino acids in the treatment of phenylketonuria (PKU). J Inherit Metab Dis 2006; 29(6): 732-8. doi: 10.1007/s10545-006-0395-8. PubMed PMID: WOS:000243365300008.
  • 25. Schindeler S, Ghosh-Jerath S, Thompson S, Rocca A, Joy P, Kemp A, et al. The effects of large neutral amino acid supplements in PKU: An MRS and neuropsychological study. Mol Genet Metab 2007; 91(1): 48-54. doi: 10.1016/j.ymgme.2007.02.002. PubMed PMID: WOS:000246540200007.
  • 26. van Spronsen FJ, de Groot MJ, Hoeksma M, Reijngoud DJ, van Rijn M. Large neutral amino acids in the treatment of PKU: from theory to practice. J Inherit Metab Dis 2010; 33(6): 671-6. doi: 10.1007/s10545-010-9216-1. PubMed PMID: WOS:000284647100006.
  • 27. Hoeksma M, Reijngoud DJ, Pruim J, de Valk HW, Paans AMJ, van Spronsen FJ. Phenylketonuria: High plasma phenylalanine decreases cerebral protein synthesis. Mol Genet Metab 2009; 96(4): 177-82. doi: 10.1016/j.ymgme.2008.12.019. PubMed PMID: WOS:000264737700005.
  • 28. Lindegren ML, Krishnaswami S, Reimschisel T, Fonnesbeck C, Sathe NA, McPheeters ML. A systematic review of BH4 (sapropterin) for the adjuvant treatment of phenylketonuria. JIMD reports 2013; 8: 109-19. doi: 10.1007/8904_2012_168. PubMed PMID: 23430527; PubMed Central PMCID: PMC3565680.
  • 29. MacDonald A, Ahring K, Dokoupil K, Gokmen-Ozel H, Lammardo AM, Motzfeldt K, ve ark., Adjusting diet with sapropterin in phenylketonuria: what factors should be considered? British J Nutr 2011; 106(2): 175-82. Epub 2011/04/07. doi: 10.1017/s0007114511000298. PubMed PMID: 21466737.
  • 30. Levy HL, Milanowski A, Chakrapani A, Cleary M, Lee P, Trefz FK, et al. Efficacy of sapropterin dihydrochloride (tetrahydrobiopterin, 6R-BH4) for reduction of phenylalanine concentration in patients with phenylketonuria: a phase III randomised placebo-controlled study. Lancet 2007; 370(9586): 504-10. doi: Doi 10.1016/S0140-6736(07)61234-3. PubMed PMID: WOS:000248717800029.
  • 31. Somaraju UR, Merrin M. Sapropterin dihydrochloride for phenylketonuria. The Cochrane Database of Systematic Reviews. 2015; 3: Cd008005. Epub 2015/03/31. doi: 10.1002/14651858.CD008005.pub4. PubMed PMID: 25812600.
  • 32. Sarkissian CN, Gamez A, Wang L, Charbonneau M, Fitzpatrick P, Lemontt JF, et al. Preclinical evaluation of multiple species of PEGylated recombinant phenylalanine ammonia lyase for the treatment of phenylketonuria. Pro Nat Acad Sci USA. 2008;105(52):20894-9. doi: 10.1073/pnas.0808421105. PubMed PMID: 19095795; PubMed Central PMCID: PMC2634911.
  • 33. Aydas SB, Ozturk S, Aslim B. Phenylalanine ammonia lyase (PAL) enzyme activity and antioxidant properties of some cyanobacteria isolates. Food Chem 2013; 136(1): 164-9. doi: 10.1016/j.foodchem.2012.07.119. PubMed PMID: WOS:000309897000024.
  • 34. Kim W, Erlandsen H, Surendran S, Stevens RC, Gamez A, Michols-Matalon K, et al. Trends in enzyme therapy for phenylketonuria. Molecular therapy : J Am Soc Gene Ther 2004; 10(2): 220-4. doi: 10.1016/j.ymthe.2004.05.001. PubMed PMID: 15294168.
  • 35. Gamez A, Sarkissian CN, Wang L, Kim W, Straub M, Patch MG, et al. Development of pegylated forms of recombinant Rhodosporidium toruloides phenylalanine ammonia-lyase for the treatment of classical phenylketonuria. Molecular therapy : J Am Soc Gene Ther 2005; 11(6): 986-9. doi: 10.1016/j.ymthe.2005.02.013. PubMed PMID: 15922970.

Nutrition and New Treatment Approaches in Phenylketonuria

Yıl 2018, Cilt: 16 Sayı: 2, 187 - 198, 01.08.2018

İzzet Ülker Nevin Şanlıer

Öz

Kaynakça

  • 1. Blau N, van Spronsen FJ, Levy HL. Phenylketonuria. Lancet 2010; 376(9750): 1417-27.
  • 2. Sarkissian CN, Gamez A, Scriver CR. What we know that could influence future treatment of phenylketonuria. J Inherit Metab Dis 2009; 32(1):3-9.
  • 3. Ozturk Y. Metabolik Hastaliklarda Beslenme. http://wwwyesimozturkcom/PDF/YesimOzturk_MetabolikHastaliklardaBeslenme 2008pdf erişim tarihi 26/02/2016.
  • 4. Köksal G. Gökmen Özel H. Metabolik hastalıklarda beslenme. Ankara; Klasmat Matbaası; 2008, p.10-11.
  • 5. Ahring K, Bélanger-Quintana A, Dokoupil K, Ozel HG, Lammardo AM, MacDonald A, et al. Dietary management practices in phenylketonuria across European centres. Clin Nutr 2009; 28(3): 231-6. doi: http://dx.doi.org/10.1016/j.clnu.2009.03.004.
  • 6. Özer I. Fenilketonüri örneğinde doğumsal metabolik hastalıklarda genel tedavi yaklaşımı. Klin Pediatr. 2004; 3: 26-30(1).
  • 7. Singh RH, Rohr F, Frazier D, Cunningham A, Mofidi S, Ogata B, et al. Recommendations for the nutrition management of phenylalanine hydroxylase deficiency. Genetics in medicine : official J Am Col Med Genet 2014; 16(2): 121-31. doi: 10.1038/gim.2013.179. PubMed PMID: 24385075; PubMed Central PMCID: PMC3918542.
  • 8. Vockley J, Andersson HC, Antshel KM, Braverman NE, Burton BK, Frazier DM, et al. Phenylalanine hydroxylase deficiency: diagnosis and management guideline. Genetics in medicine : official journal of the American College of Medical Genetics. 2014;16(2):188-200. Epub 2014/01/05. doi: 10.1038/gim.2013.157. PubMed PMID: 24385074.
  • 9. Cunningham A, Bausell H, Brown M, Chapman M, DeFouw K, Ernst S, et al. Recommendations for the use of sapropterin in phenylketonuria. Mol Genet Metab 2012; 106(3): 269-76. doi: 10.1016/j.ymgme.2012.04.004. PubMed PMID: WOS:000305818600003.
  • 10. National Institutes of Health Consensus Development P. National Institutes of Health Consensus Development Conference Statement: phenylketonuria: screening and management, October 16-18, 2000. Pediatrics. 2001; 108(4): 972-82. PubMed PMID: 11581453.
  • 11. Ney DM, Blank RD, Hansen KE. Advances in the nutritional and pharmacological management of phenylketonuria. Curr Op Clin Nutr Metab Care 2014; 17(1): 61-8. doi: 10.1097/MCO.0000000000000002. PubMed PMID: 24136088; PubMed Central PMCID: PMC4004170.
  • 12. Hvas AM, Nexo E, Nielsen JB. Vitamin B12 and vitamin B6 supplementation is needed among adults with phenylketonuria (PKU). J Inherit Metab Dis 2006; 29(1): 47-53. Epub 2006/04/08. doi: 10.1007/s10545-006-0108-3. PubMed PMID: 16601867.
  • 13. Moseley K, Koch R, Moser AB. Lipid status and long-chain polyunsaturated fatty acid concentrations in adults and adolescents with phenylketonuria on phenylalanine-restricted diet. J Inherit Metab Dis 2002;25(1):56-64.
  • 14. Üstüner Top F. The difficulties of families of children with phenylketonuria: A qualitative study. HEAD 2015; 12(1): 62-8. doi: doi:10.5222/HEAD.2015.062.
  • 15. Blau N BP. Disorders of phenylalanine and tetrahydrobiopterin metabolism. Hoffmann Jl CJ, In: Physician's Guide to the Diagnosis, Treatment, and Follow-Up of Inherited Metabolic Diseases, Berlin Heidelberg 2006, p. 26-34.
  • 16. MacDonald A, Gokmen-Ozel H, van Rijn M, Burgard P. The reality of dietary compliance in the management of phenylketonuria. J Inherit Metab Dis 2010; 33(6): 665-70. Epub 2010/04/08. doi: 10.1007/s10545-010-9073-y. PubMed PMID: 20373144.
  • 17. Fuhrman MP, Galvin TA, Ireton-Jones CS, Thorpe J. Practice paper of the American Dietetic Association: Home care--opportunities for food and nutrition professionals. J Am Dietet Ass 2009; 109(6): 1092-100. Epub 2009/06/02. PubMed PMID: 19480085.
  • 18. Bakırel NA. Okul öncesi çağı klasik fenilketonürili çocukların malnütrisyon durumunun saptanması (Yüksek Lisans Tezi). Ankara: Gazi Üniversitesi; 2008.
  • 19. Etzel MR. Manufacture and use of dairy protein fractions. J Nutr 2004; 134(4): 996s-1002s. PubMed PMID: WOS:000220681700047.
  • 20. Bruck WM, Redgrave M, Tuohy KM, Lonnerdal B, Graverholt G, Hernell O, et al. Effects of bovine alpha-lactalbumin and casein glycomacropeptide-enriched infant formulae on faecal microbiota in healthy term infants. J Pediatr Gastr Nutr 2006; 43(5): 673-9. PubMed PMID: WOS:000241906800022.
  • 21. van Calcar SC, Ney DM. Food products made with glycomacropeptide, a low-phenylalanine whey protein, provide a new alternative to amino acid-based medical ffoods for nutrition management ofphenylketonuria. J Acad Nutr Diet 2012; 112(8): 1201-10. doi: 10.1016/j.jand.2012.05.004. PubMed PMID: WOS:000307318100011.
  • 22. Dangin M, Boirie Y, Garcia-Rodenas C, Gachon P, Fauquant J, Callier P, et al. The digestion rate of protein is an independent regulating factor of postprandial protein retention. Am J Physiol-Endoc M 2001; 280(2): E340-E8. PubMed PMID: WOS:000166453400020.
  • 23. Calbet JAL, MacLean DA. Plasma glucagon and insulin responses depend on the rate of appearance of amino acids after ingestion of different protein solutions in humans. J Nutr 2002; 132(8): 2174-82. PubMed PMID: WOS:000177398400010.
  • 24. Matalon R, Michals-Matalon K, Bhatia G, Grechanina E, Novikov P, McDonald JD, et al. Large neutral amino acids in the treatment of phenylketonuria (PKU). J Inherit Metab Dis 2006; 29(6): 732-8. doi: 10.1007/s10545-006-0395-8. PubMed PMID: WOS:000243365300008.
  • 25. Schindeler S, Ghosh-Jerath S, Thompson S, Rocca A, Joy P, Kemp A, et al. The effects of large neutral amino acid supplements in PKU: An MRS and neuropsychological study. Mol Genet Metab 2007; 91(1): 48-54. doi: 10.1016/j.ymgme.2007.02.002. PubMed PMID: WOS:000246540200007.
  • 26. van Spronsen FJ, de Groot MJ, Hoeksma M, Reijngoud DJ, van Rijn M. Large neutral amino acids in the treatment of PKU: from theory to practice. J Inherit Metab Dis 2010; 33(6): 671-6. doi: 10.1007/s10545-010-9216-1. PubMed PMID: WOS:000284647100006.
  • 27. Hoeksma M, Reijngoud DJ, Pruim J, de Valk HW, Paans AMJ, van Spronsen FJ. Phenylketonuria: High plasma phenylalanine decreases cerebral protein synthesis. Mol Genet Metab 2009; 96(4): 177-82. doi: 10.1016/j.ymgme.2008.12.019. PubMed PMID: WOS:000264737700005.
  • 28. Lindegren ML, Krishnaswami S, Reimschisel T, Fonnesbeck C, Sathe NA, McPheeters ML. A systematic review of BH4 (sapropterin) for the adjuvant treatment of phenylketonuria. JIMD reports 2013; 8: 109-19. doi: 10.1007/8904_2012_168. PubMed PMID: 23430527; PubMed Central PMCID: PMC3565680.
  • 29. MacDonald A, Ahring K, Dokoupil K, Gokmen-Ozel H, Lammardo AM, Motzfeldt K, ve ark., Adjusting diet with sapropterin in phenylketonuria: what factors should be considered? British J Nutr 2011; 106(2): 175-82. Epub 2011/04/07. doi: 10.1017/s0007114511000298. PubMed PMID: 21466737.
  • 30. Levy HL, Milanowski A, Chakrapani A, Cleary M, Lee P, Trefz FK, et al. Efficacy of sapropterin dihydrochloride (tetrahydrobiopterin, 6R-BH4) for reduction of phenylalanine concentration in patients with phenylketonuria: a phase III randomised placebo-controlled study. Lancet 2007; 370(9586): 504-10. doi: Doi 10.1016/S0140-6736(07)61234-3. PubMed PMID: WOS:000248717800029.
  • 31. Somaraju UR, Merrin M. Sapropterin dihydrochloride for phenylketonuria. The Cochrane Database of Systematic Reviews. 2015; 3: Cd008005. Epub 2015/03/31. doi: 10.1002/14651858.CD008005.pub4. PubMed PMID: 25812600.
  • 32. Sarkissian CN, Gamez A, Wang L, Charbonneau M, Fitzpatrick P, Lemontt JF, et al. Preclinical evaluation of multiple species of PEGylated recombinant phenylalanine ammonia lyase for the treatment of phenylketonuria. Pro Nat Acad Sci USA. 2008;105(52):20894-9. doi: 10.1073/pnas.0808421105. PubMed PMID: 19095795; PubMed Central PMCID: PMC2634911.
  • 33. Aydas SB, Ozturk S, Aslim B. Phenylalanine ammonia lyase (PAL) enzyme activity and antioxidant properties of some cyanobacteria isolates. Food Chem 2013; 136(1): 164-9. doi: 10.1016/j.foodchem.2012.07.119. PubMed PMID: WOS:000309897000024.
  • 34. Kim W, Erlandsen H, Surendran S, Stevens RC, Gamez A, Michols-Matalon K, et al. Trends in enzyme therapy for phenylketonuria. Molecular therapy : J Am Soc Gene Ther 2004; 10(2): 220-4. doi: 10.1016/j.ymthe.2004.05.001. PubMed PMID: 15294168.
  • 35. Gamez A, Sarkissian CN, Wang L, Kim W, Straub M, Patch MG, et al. Development of pegylated forms of recombinant Rhodosporidium toruloides phenylalanine ammonia-lyase for the treatment of classical phenylketonuria. Molecular therapy : J Am Soc Gene Ther 2005; 11(6): 986-9. doi: 10.1016/j.ymthe.2005.02.013. PubMed PMID: 15922970.
Toplam 35 adet kaynakça vardır.

Ayrıntılar

Birincil Dil Türkçe
Konular İç Hastalıkları
Bölüm Derleme
Yazarlar

İzzet Ülker

Nevin Şanlıer Bu kişi benim

Yayımlanma Tarihi 1 Ağustos 2018
Yayımlandığı Sayı Yıl 2018 Cilt: 16 Sayı: 2

Kaynak Göster

APA Ülker, İ., & Şanlıer, N. (2018). Fenilketonüride Beslenme ve Yeni Tedavi Yaklaşımları. Güncel Pediatri, 16(2), 187-198.
AMA Ülker İ, Şanlıer N. Fenilketonüride Beslenme ve Yeni Tedavi Yaklaşımları. Güncel Pediatri. Ağustos 2018;16(2):187-198.
Chicago Ülker, İzzet, ve Nevin Şanlıer. “Fenilketonüride Beslenme Ve Yeni Tedavi Yaklaşımları”. Güncel Pediatri 16, sy. 2 (Ağustos 2018): 187-98.
EndNote Ülker İ, Şanlıer N (01 Ağustos 2018) Fenilketonüride Beslenme ve Yeni Tedavi Yaklaşımları. Güncel Pediatri 16 2 187–198.
IEEE İ. Ülker ve N. Şanlıer, “Fenilketonüride Beslenme ve Yeni Tedavi Yaklaşımları”, Güncel Pediatri, c. 16, sy. 2, ss. 187–198, 2018.
ISNAD Ülker, İzzet - Şanlıer, Nevin. “Fenilketonüride Beslenme Ve Yeni Tedavi Yaklaşımları”. Güncel Pediatri 16/2 (Ağustos 2018), 187-198.
JAMA Ülker İ, Şanlıer N. Fenilketonüride Beslenme ve Yeni Tedavi Yaklaşımları. Güncel Pediatri. 2018;16:187–198.
MLA Ülker, İzzet ve Nevin Şanlıer. “Fenilketonüride Beslenme Ve Yeni Tedavi Yaklaşımları”. Güncel Pediatri, c. 16, sy. 2, 2018, ss. 187-98.
Vancouver Ülker İ, Şanlıer N. Fenilketonüride Beslenme ve Yeni Tedavi Yaklaşımları. Güncel Pediatri. 2018;16(2):187-98.
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