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The Current Information in Nutrition Therapy of Phenylketonuria

Yıl 2020, Sayı: 18, 755 - 761, 15.04.2020
https://doi.org/10.31590/ejosat.693556

Öz

Phenylketonuria (PKU) is one of the most common congenital metabolism disorders. In this disease, due to the defect in the phenylalanine hydroxylase enzyme made in the liver, phenylalanine cannot follow the tyrosine pathway and the symptoms of phenylalanine metabolites occur in body tissues, organs and body fluids. Nutritional therapy is applied in the treatment of the disease depending on the type of PKU. The purpose of nutritional therapy is to minimize the amount of phenylalane taken with nutrients and to keep the level of blood phenylale within normal limits. For this reason, a special nutrition program is applied throughout the life from the newborn period. The first step in the diagnosis of PKU is to determine the level of plasma phenylalanine with the blood sample taken from the heel of the newborn. Different types of hyperphenylalaninemias are seen as a result of phenylalanine hydroxylase enzyme and BH4 metabolism disorder. In the formation of BH4 cofactor defects, there is a defect in the metabolism of tetrahydrobiopterin, and its findings are different from classical PKU.In addition to the proper nutrition program, LNAA and sapropterin (a synthetic form of BH4) are used in the treatment, according to the results of new studies. In classical PKU, when there is no medical nutrition treatment specific to the disease, many clinical findings such as mental retardation, skin-hair pigmentation disorders, growth retardation, microcephaly, epilepsy, behavioral disorders, hyperactivity and anxiety can be seen.In order for the symptoms associated with PKU to decrease and individuals with PKU to live a healthier life, they should comply with dietary treatment that includes protein-restricted, high-fat and carbohydrate-containing foods Except for vegetables and fruits limited from phenylalanine, essential protein sources should be provided with special formulas with added tyrosine and essential amino acids and foods enriched with vitamins and minerals that may be deficient. However, the implementation of an effective nutrition therapy is possible by providing communication between the healthcare team and the family and introducing the disease to the family, and training in the development and treatment of the disease.

Kaynakça

  • Ahring, K., Bélanger-Quintana, A., Dokoupil, K., Gokmen Ozel, H., Lammardo, A. M., MacDonald, A., Motzfeldt, K., Nowacka, M., Robert, M., van Rijn, M., (2009). Dietary Management Practices in Phenylketonuria Across European Centres. Clinical Nutrition, 28(3), 231–236.
  • Azaripour, A., Abbasi, H., 2020. Effect of Type and Amount of Modified Corn Starches on Qualitative Properties of Low-Protein Biscuits for Phenylketonuria. Food Sci Nutr.8:281–290.
  • Bay, S. (2019). Bölgemizdeki Fenilketonüri Hastalığının ve Tedavisinin Ailelerin ve Fenilketonürili Çocukların Yaşam Kaliteleri Üzerine Olan Etkisinin Araştırılması (Uzmanlık Tezi). Akdeniz Üniversitesi, Antalya.
  • Blau, N., van Spronsen, FJ., Levy, HL., 2010. Phenylketonuria. Lancet, 376:1417–27.
  • Concolino, D., Mascaro, I., Moricca, M.T., Bonapace, G., Matalon, K., Trapasso, J., Radhakrishnan, G., Ferrara, C., Matalon, R., Strisciuglio, P., 2017. Long-Term Treatment of Phenylketonuria with a New Medical Food Containing Large Neutral Amino Acids. Eur J Clin Nutr, 71, 51–55.
  • Demirdas, S., Coakley, K.E., Bisschop, P.H., Hollak, C.E.M., Bosch, A.M., Singh, R.H.,2015. Bone Health in Phenylketonuria: A Systematic Review and Meta-analysis. Orphanet Journal of Rare Diseases, 10:17.
  • Deon, M., Landgraf, S.S., Lamberty, J.F., Moura, D.J., Saffi, J., Wajner, M., Vargas, C.R., 2015. Protective Effect of L-Carnitine On Phenylalanine-İnduced DNA Damage. Metab Brain Dis, 30, 925–933.
  • Douglas T.D., Nucci, A.M., Berry, A.M., Henes, S.T., Singh, R.H., 2019. Large Neutral Amino Acid Status in Association with P:T Ratio and Diet in Adult and Pediatric Patients With Phenylketonuria. JIMD Reports.;50:50–59.
  • Durrer, KE, Allen, MS, von Herbing I.H., (2017) Genetically Engineered Probiotic for The Treatment of Phenylketonuria (PKU); Assessment of a Novel Treatment in Vitro and in The Pahenu2 Mouse Model of PKU. PLoS ONE 12(5): e0176286.
  • Enacán, R.E., Miñana, M.N., Fernandez, L., Valle, M.G., Salerno, M., Fraga, C.I., Santos-Simarro, F., Prieto, L., Lapunzina, P., Specola, N., Chiesa, A.N., 2019. Phenylalanine Hydroxylase (PAH) Genotyping in PKU Argentine Patients. Journal of Inborn Errors of Metabolism & Screening, Volume 7: e20190012.
  • Evans, S., Daly, A., Chahal, S., MacDonald, J., & MacDonald, A. (2015). Food Acceptance and Neophobia in Children with Phenylketonuria: A Prospective Controlled Study. Journal of Human Nutrition and Dietetics, 29(4), 427–433.
  • Evans, S., Daly, A., Wildgoose, J., Cochrane, B., Chahal, S., Ashmore, C., Loveridge, N., MacDonald, A., (2019). Growth, Protein and Energy Intake in Children with PKU Taking a Weaning Protein Substitute in the First Two Years of Life: A Case-Control Study. Nutrients, 11, 552.
  • Herenger, Y., Maes, E., François, L., Pasco, J., Bouchereau, J., Pichard, S., de Baulny, H.O., Schiff, M., 2019. Determining factors of the Cognitive Outcome in Early Treated PKU: A Study of 39 Pediatric Patients. Molecular Genetics and Metabolism Reports 20:1004983.
  • Hoeksma, M., Reijngoud, D.J., Pruim, J., de Valk, H.W., Paans, A.M., vanSpronsen FJ., 2009. Phenylketonuria: High Plasma Phenylalanine Decreases Cerebral Protein Synthesis. Mol Genet Metab, 96:177–82.
  • http://www.turkomp.gov.tr/: retrieved 23.01.2020
  • Kose, E., Aksoy, B., Kuyum, P., Tuncer, N., Arslan, N., Ozturk, Y. (2018). The Effects of Breastfeeding in Infants with Phenylketonuria. Journal of Pediatric Nursing, 38, 27–32.
  • Kose, E., Arslan, N., 2019. Vitamin/mineral and Micronutrient Status in Patients with Classical Phenylketonuria. Clinical Nutrition, Volume 38, Issue 1.
  • Köksal, G., Gökmen Özel, H., 2019. Çocuk Hastalıklarında Beslenme Tedavisi. Hatiboğlu Yayınevi.
  • Levy, H., Lamppu, D., Anastosoaie, V., Baker, J.L., DiBona, K., Hawthorne, S., Lindenberger, J., Kinch, D., Seymour, A., McIlduff, M., Watling, S., Vockley, J., 2019. 5-Year Retrospective Analysis of Patients with Phenylketonuria (PKU) and Hyperphenylalaninemia Treated at Two Specialized Clinics. Molecular Genetics and Metabolism, https://doi.org/10.1016/j.ymgme.2019.12.007
  • Lichter-Konecki, U., Vockley, J., 2019. Phenylketonuria: Current Treatments and Future Developments. Drugs 79, 495–500.
  • Lindegren M.L., Krishnaswami S., Reimschisel T., Fonnesbeck C., Sathe N.A., McPheeters M.L. (2012) A Systematic Review of BH4 (Sapropterin) for the Adjuvant Treatment of Phenylketonuria. JIMD Reports- Case and Research Reports, 109-119.
  • Liu, N., Huang, Q., Li, Q. Zhao, D., Li, X., Cui, L., Bai, Y., Feng, Y., Kong, X., 2017. Spectrum of PAH Gene Variants Among a Population of Han Chinese Patients with Phenylketonuria from Northern China. BMC Med Genet, 18, 108.
  • Mahan, LK., Raymond, JL., 2016. Krause’s Food & the Nutrition Care Process [14 ed.].
  • McPheeters, M.L., Lindegren, M.L., Sathe, N., Reimschisel, T., 2012. Adjuvant Treatment for Phenylketonuria: Future Research Needs: Identification of Future Research Needs from Comparative Effectiveness Review No. 56 [Internet], Rockville, Md, Agency for Healthcare Research and Quality (US).
  • Mitchell, J.J, Trakadis, Y.J, Scriver, C.R., 2011. Phenylalanine Hydroxylase Deficiency. Genet Med;13(8):697-707.
  • Ney, D.M, Gleason, S.T, van Calcar, S.C., MacLeod, E.L., Nelson, K.L., Etzel, M.R., Rice, G.M., Wolff, J.A., 2009. Nutritional Management of PKU with Glycomacropeptide from Cheese Whey. J Inherit Metab Dis, 32:32-39.
  • Ney, D.M., Blank, R.D., Hansen, K.E., 2014. Advances in the Nutritional and Pharmacological Management of Phenylketonuria. Curr Opin Clin Nutr Metab Care, 17(1): 61–68.
  • O’Sullivan, A.J., Pigat, S., O’Mahony, C., Gibney, M. J., McKevitt, A. I., 2016. Probabilistic Modelling to Assess Exposure to Three Artificial Sweeteners of Young Irish Patients Aged 1–3 Years with PKU and CMPA. Food Additives & Contaminants: Part A, 33(11), 1660–1671.
  • Peate, Ian. (2020). Blood Spot Screening Programme for Newborns. British Journal of Healthcare Assistants Vol. 14, No.1.
  • Pena, M.J., Almeida, M.F., van Dam, E. Ahring, K., Bélanger-Quintana, A., Dokoupil, K., Gokmen-Ozel, H., Lammardo, A.M., MacDonald, A., Robert, M., Rocha, J.C., 2015. Special Low Protein Foods for Phenylketonuria: Availability in Europe and an Examination of Their Nutritional Profile. Orphanet J Rare Dis 10, 162.
  • Rocha, J.C., MacDonald, A., 2016. Dietary İntervention in the Management of Phenylketonuria: Current Perspectives. Pediatric Health Med Ther.; 7: 155–163.
  • Strisciuglio, P., Concolino, D., 2014. New Strategies for the Treatment of Phenylketonuria (PKU). Metabolites, 4, 1007-1017.
  • Sur Ünal, Ü., Çifçili, S.S., 2019. Emzirmede Bebek Açısından Kontrendikasyon Oluşturan Durumlar. Telatar B, editör. Aile Hekimliğinde Anne Sütünün Anne ve Bebek Sağlığı Açısından Önemi. 1. Baskı. Ankara: Türkiye Klinikleri; p.93-6.
  • Van Spronsen, F. J., van Wegberg, A.M.J., Ahring, K., Bélanger-Quintana, A., Blau, N., Bosch, A. M., Burlina, A., Campistol, J., Feillet, F., Gizewska, M., Huijbregts, S.C., Kearney, S., Leuzzi, V., Maillot, F., Muntau, A.C., Trefz, F.K., van rijn, M., Walter, J.H., MacDonald, A. 2017. Key European Guidelines for the Diagnosis and Management of Patients with Phenylketonuria. The Lancet Diabetes & Endocrinology, 5(9), 743–756.
  • Van Wegberg, A.M.J., Evers, R.A.F., van Dam, E., de Vries, M.C., Janssen, M.C.H., Heiner-Fokkema, M.R., van Spronsen, F.J., 2019. Does the 48-hour BH4 loading test miss responsive PKU patients? Molecular Genetics and Metabolism., https://doi.org/10.1016/j.ymgme.2019.12.011.
  • Van Vliet, D., Annemiek, M.J., van Wegberg, Ahring, K., Bik-Multanowski, M., Casas, K., Didycz, B., Djordjevic, M., Hertecant, J.L., Leuzzi, V., Mathisen, P., Nardecchia, F., Powell, K.K., Rutsch, F., Stojiljkovic, M., Trefz, F.K., Usurelu, N., Wilson, C., van Karnebeek, C.D., Hanley, W.B., van Spronsen, F.J., 2019. Untreated PKU Patients without Intellectual Disability: What Do They Teach Us? Nutrients, 11, 2572.

The Current Information in Nutrition Therapy of Phenylketonuria

Yıl 2020, Sayı: 18, 755 - 761, 15.04.2020
https://doi.org/10.31590/ejosat.693556

Öz

Phenylketonuria (PKU) is one of the most common congenital metabolism disorders. In this disease, due to the defect in the phenylalanine hydroxylase enzyme made in the liver, phenylalanine cannot follow the tyrosine pathway and the symptoms of phenylalanine metabolites occur in body tissues, organs and body fluids. Nutritional therapy is applied in the treatment of the disease depending on the type of PKU. The purpose of nutritional therapy is to minimize the amount of phenylalane taken with nutrients and to keep the level of blood phenylale within normal limits. For this reason, a special nutrition program is applied throughout the life from the newborn period. The first step in the diagnosis of PKU is to determine the level of plasma phenylalanine with the blood sample taken from the heel of the newborn. Different types of hyperphenylalaninemias are seen as a result of phenylalanine hydroxylase enzyme and BH4 metabolism disorder. In the formation of BH4 cofactor defects, there is a defect in the metabolism of tetrahydrobiopterin, and its findings are different from classical PKU.In addition to the proper nutrition program, LNAA and sapropterin (a synthetic form of BH4) are used in the treatment, according to the results of new studies. In classical PKU, when there is no medical nutrition treatment specific to the disease, many clinical findings such as mental retardation, skin-hair pigmentation disorders, growth retardation, microcephaly, epilepsy, behavioral disorders, hyperactivity and anxiety can be seen.In order for the symptoms associated with PKU to decrease and individuals with PKU to live a healthier life, they should comply with dietary treatment that includes protein-restricted, high-fat and carbohydrate-containing foods Except for vegetables and fruits limited from phenylalanine, essential protein sources should be provided with special formulas with added tyrosine and essential amino acids and foods enriched with vitamins and minerals that may be deficient. However, the implementation of an effective nutrition therapy is possible by providing communication between the healthcare team and the family and introducing the disease to the family, and training in the development and treatment of the disease.

Kaynakça

  • Ahring, K., Bélanger-Quintana, A., Dokoupil, K., Gokmen Ozel, H., Lammardo, A. M., MacDonald, A., Motzfeldt, K., Nowacka, M., Robert, M., van Rijn, M., (2009). Dietary Management Practices in Phenylketonuria Across European Centres. Clinical Nutrition, 28(3), 231–236.
  • Azaripour, A., Abbasi, H., 2020. Effect of Type and Amount of Modified Corn Starches on Qualitative Properties of Low-Protein Biscuits for Phenylketonuria. Food Sci Nutr.8:281–290.
  • Bay, S. (2019). Bölgemizdeki Fenilketonüri Hastalığının ve Tedavisinin Ailelerin ve Fenilketonürili Çocukların Yaşam Kaliteleri Üzerine Olan Etkisinin Araştırılması (Uzmanlık Tezi). Akdeniz Üniversitesi, Antalya.
  • Blau, N., van Spronsen, FJ., Levy, HL., 2010. Phenylketonuria. Lancet, 376:1417–27.
  • Concolino, D., Mascaro, I., Moricca, M.T., Bonapace, G., Matalon, K., Trapasso, J., Radhakrishnan, G., Ferrara, C., Matalon, R., Strisciuglio, P., 2017. Long-Term Treatment of Phenylketonuria with a New Medical Food Containing Large Neutral Amino Acids. Eur J Clin Nutr, 71, 51–55.
  • Demirdas, S., Coakley, K.E., Bisschop, P.H., Hollak, C.E.M., Bosch, A.M., Singh, R.H.,2015. Bone Health in Phenylketonuria: A Systematic Review and Meta-analysis. Orphanet Journal of Rare Diseases, 10:17.
  • Deon, M., Landgraf, S.S., Lamberty, J.F., Moura, D.J., Saffi, J., Wajner, M., Vargas, C.R., 2015. Protective Effect of L-Carnitine On Phenylalanine-İnduced DNA Damage. Metab Brain Dis, 30, 925–933.
  • Douglas T.D., Nucci, A.M., Berry, A.M., Henes, S.T., Singh, R.H., 2019. Large Neutral Amino Acid Status in Association with P:T Ratio and Diet in Adult and Pediatric Patients With Phenylketonuria. JIMD Reports.;50:50–59.
  • Durrer, KE, Allen, MS, von Herbing I.H., (2017) Genetically Engineered Probiotic for The Treatment of Phenylketonuria (PKU); Assessment of a Novel Treatment in Vitro and in The Pahenu2 Mouse Model of PKU. PLoS ONE 12(5): e0176286.
  • Enacán, R.E., Miñana, M.N., Fernandez, L., Valle, M.G., Salerno, M., Fraga, C.I., Santos-Simarro, F., Prieto, L., Lapunzina, P., Specola, N., Chiesa, A.N., 2019. Phenylalanine Hydroxylase (PAH) Genotyping in PKU Argentine Patients. Journal of Inborn Errors of Metabolism & Screening, Volume 7: e20190012.
  • Evans, S., Daly, A., Chahal, S., MacDonald, J., & MacDonald, A. (2015). Food Acceptance and Neophobia in Children with Phenylketonuria: A Prospective Controlled Study. Journal of Human Nutrition and Dietetics, 29(4), 427–433.
  • Evans, S., Daly, A., Wildgoose, J., Cochrane, B., Chahal, S., Ashmore, C., Loveridge, N., MacDonald, A., (2019). Growth, Protein and Energy Intake in Children with PKU Taking a Weaning Protein Substitute in the First Two Years of Life: A Case-Control Study. Nutrients, 11, 552.
  • Herenger, Y., Maes, E., François, L., Pasco, J., Bouchereau, J., Pichard, S., de Baulny, H.O., Schiff, M., 2019. Determining factors of the Cognitive Outcome in Early Treated PKU: A Study of 39 Pediatric Patients. Molecular Genetics and Metabolism Reports 20:1004983.
  • Hoeksma, M., Reijngoud, D.J., Pruim, J., de Valk, H.W., Paans, A.M., vanSpronsen FJ., 2009. Phenylketonuria: High Plasma Phenylalanine Decreases Cerebral Protein Synthesis. Mol Genet Metab, 96:177–82.
  • http://www.turkomp.gov.tr/: retrieved 23.01.2020
  • Kose, E., Aksoy, B., Kuyum, P., Tuncer, N., Arslan, N., Ozturk, Y. (2018). The Effects of Breastfeeding in Infants with Phenylketonuria. Journal of Pediatric Nursing, 38, 27–32.
  • Kose, E., Arslan, N., 2019. Vitamin/mineral and Micronutrient Status in Patients with Classical Phenylketonuria. Clinical Nutrition, Volume 38, Issue 1.
  • Köksal, G., Gökmen Özel, H., 2019. Çocuk Hastalıklarında Beslenme Tedavisi. Hatiboğlu Yayınevi.
  • Levy, H., Lamppu, D., Anastosoaie, V., Baker, J.L., DiBona, K., Hawthorne, S., Lindenberger, J., Kinch, D., Seymour, A., McIlduff, M., Watling, S., Vockley, J., 2019. 5-Year Retrospective Analysis of Patients with Phenylketonuria (PKU) and Hyperphenylalaninemia Treated at Two Specialized Clinics. Molecular Genetics and Metabolism, https://doi.org/10.1016/j.ymgme.2019.12.007
  • Lichter-Konecki, U., Vockley, J., 2019. Phenylketonuria: Current Treatments and Future Developments. Drugs 79, 495–500.
  • Lindegren M.L., Krishnaswami S., Reimschisel T., Fonnesbeck C., Sathe N.A., McPheeters M.L. (2012) A Systematic Review of BH4 (Sapropterin) for the Adjuvant Treatment of Phenylketonuria. JIMD Reports- Case and Research Reports, 109-119.
  • Liu, N., Huang, Q., Li, Q. Zhao, D., Li, X., Cui, L., Bai, Y., Feng, Y., Kong, X., 2017. Spectrum of PAH Gene Variants Among a Population of Han Chinese Patients with Phenylketonuria from Northern China. BMC Med Genet, 18, 108.
  • Mahan, LK., Raymond, JL., 2016. Krause’s Food & the Nutrition Care Process [14 ed.].
  • McPheeters, M.L., Lindegren, M.L., Sathe, N., Reimschisel, T., 2012. Adjuvant Treatment for Phenylketonuria: Future Research Needs: Identification of Future Research Needs from Comparative Effectiveness Review No. 56 [Internet], Rockville, Md, Agency for Healthcare Research and Quality (US).
  • Mitchell, J.J, Trakadis, Y.J, Scriver, C.R., 2011. Phenylalanine Hydroxylase Deficiency. Genet Med;13(8):697-707.
  • Ney, D.M, Gleason, S.T, van Calcar, S.C., MacLeod, E.L., Nelson, K.L., Etzel, M.R., Rice, G.M., Wolff, J.A., 2009. Nutritional Management of PKU with Glycomacropeptide from Cheese Whey. J Inherit Metab Dis, 32:32-39.
  • Ney, D.M., Blank, R.D., Hansen, K.E., 2014. Advances in the Nutritional and Pharmacological Management of Phenylketonuria. Curr Opin Clin Nutr Metab Care, 17(1): 61–68.
  • O’Sullivan, A.J., Pigat, S., O’Mahony, C., Gibney, M. J., McKevitt, A. I., 2016. Probabilistic Modelling to Assess Exposure to Three Artificial Sweeteners of Young Irish Patients Aged 1–3 Years with PKU and CMPA. Food Additives & Contaminants: Part A, 33(11), 1660–1671.
  • Peate, Ian. (2020). Blood Spot Screening Programme for Newborns. British Journal of Healthcare Assistants Vol. 14, No.1.
  • Pena, M.J., Almeida, M.F., van Dam, E. Ahring, K., Bélanger-Quintana, A., Dokoupil, K., Gokmen-Ozel, H., Lammardo, A.M., MacDonald, A., Robert, M., Rocha, J.C., 2015. Special Low Protein Foods for Phenylketonuria: Availability in Europe and an Examination of Their Nutritional Profile. Orphanet J Rare Dis 10, 162.
  • Rocha, J.C., MacDonald, A., 2016. Dietary İntervention in the Management of Phenylketonuria: Current Perspectives. Pediatric Health Med Ther.; 7: 155–163.
  • Strisciuglio, P., Concolino, D., 2014. New Strategies for the Treatment of Phenylketonuria (PKU). Metabolites, 4, 1007-1017.
  • Sur Ünal, Ü., Çifçili, S.S., 2019. Emzirmede Bebek Açısından Kontrendikasyon Oluşturan Durumlar. Telatar B, editör. Aile Hekimliğinde Anne Sütünün Anne ve Bebek Sağlığı Açısından Önemi. 1. Baskı. Ankara: Türkiye Klinikleri; p.93-6.
  • Van Spronsen, F. J., van Wegberg, A.M.J., Ahring, K., Bélanger-Quintana, A., Blau, N., Bosch, A. M., Burlina, A., Campistol, J., Feillet, F., Gizewska, M., Huijbregts, S.C., Kearney, S., Leuzzi, V., Maillot, F., Muntau, A.C., Trefz, F.K., van rijn, M., Walter, J.H., MacDonald, A. 2017. Key European Guidelines for the Diagnosis and Management of Patients with Phenylketonuria. The Lancet Diabetes & Endocrinology, 5(9), 743–756.
  • Van Wegberg, A.M.J., Evers, R.A.F., van Dam, E., de Vries, M.C., Janssen, M.C.H., Heiner-Fokkema, M.R., van Spronsen, F.J., 2019. Does the 48-hour BH4 loading test miss responsive PKU patients? Molecular Genetics and Metabolism., https://doi.org/10.1016/j.ymgme.2019.12.011.
  • Van Vliet, D., Annemiek, M.J., van Wegberg, Ahring, K., Bik-Multanowski, M., Casas, K., Didycz, B., Djordjevic, M., Hertecant, J.L., Leuzzi, V., Mathisen, P., Nardecchia, F., Powell, K.K., Rutsch, F., Stojiljkovic, M., Trefz, F.K., Usurelu, N., Wilson, C., van Karnebeek, C.D., Hanley, W.B., van Spronsen, F.J., 2019. Untreated PKU Patients without Intellectual Disability: What Do They Teach Us? Nutrients, 11, 2572.
Toplam 36 adet kaynakça vardır.

Ayrıntılar

Birincil Dil İngilizce
Konular Mühendislik
Bölüm Makaleler
Yazarlar

Sabiha Zeynep Aydenk Köseoğlu 0000-0001-7936-8462

Seda Çelikel 0000-0001-6578-9805

Yayımlanma Tarihi 15 Nisan 2020
Yayımlandığı Sayı Yıl 2020 Sayı: 18

Kaynak Göster

APA Köseoğlu, S. Z. A., & Çelikel, S. (2020). The Current Information in Nutrition Therapy of Phenylketonuria. Avrupa Bilim Ve Teknoloji Dergisi(18), 755-761. https://doi.org/10.31590/ejosat.693556