Determination of Physicochemical, Rheological, Microbiological and Sensory Properties of Low Protein Yoghurt Substitutes Produced for PKU (Phenylketonuria) Patients

Abstract

Treatment of PKU (Phenylketonuria) is a lifelong special diet program starting from the newborn period. The aim of this study was to produce yoghurt substitute for PKU patients. A commercial low protein milk substitute, xanthan gum (1%), commercial yoghurt gelling agent (1.5%), starch (4%) and pectin (1.6%) were used to produce yoghurt substitute. Control yoghurt was produced from cow's milk. The fermentation of all samples was completed at the end of the 5th hour. The pH of the samples decreased during storage. The total solid matter of the corn starch and pectin added samples were higher than the those of others. Syneresis values of the samples with xanthan gum, pectin and commercial gelling agent were negligible. The shear stress values of xanthan gum, commercial gelling agent and starch added samples were found close to each other. The shear stress of the pectin added sample was the highest among the samples. L* values of the control and starch added samples, a* and b* values of pectin added sample were higher than those of other samples. The amount of protein and phenylalanine was higher in the pectin added sample than the other samples containing gelling. However, their values in all yoghurt substitute samples were found to be well below the upper limit value that can be consumed. Although the amount of phenylalanine tolerated in the body varies according to age, gender, weight and the degree of phenylketonuria, it is stated that up to 1000 mg per day. While numbers of Lactobacillus delbrueckii ssp. bulgaricus increased during storage, numbers of Streptococcus thermophilus increased by pectin addition and decreased in other samples. In terms of general acceptability in sensory analyses, the most preferred sample was the sample added containing commercial gelling agent. This sample was followed by the samples with corn starch and pectin. It was concluded that these yoghurt substitutes could support the missing alternative product range for the patients.

Keywords

• Phenylketonuria
• PKU patients
• Hydrocolloid
• Stabilizer
• Milk substitute

References

1. Abdel-Salam A M & Effat L K (2010). Preparation and evaluation of a novel therapeutic dairy-based drink for phenylketonuria. North American Journal of Medical Science 2 (2): 66–70
2. Acosta P & Yanicelli S (2001). Protocol 1-Phenylketonuria (PKU). In: Division R P (Ed.), The Ross Metabolic Formula System Nutrition Support Protocols, 4th ed. Columbus, Ohio: Ross Laboratories, Ross Product Division. Ahring K, Bélanger-Quintanaa 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
3. Akış M (2012). Sağlıklı ve Fenilketonürili Çocuklarda Fonksiyonel B12 Vitamin Eksikliğinin Plazma Metilmalonik Asit ve Homosistein Düzeyleri İle Araştırılması, Yüksek Lisans Tezi, Dokuz Eylül Üniversitesi Sağlık Bilimleri Enstitüsi, İzmir.
4. Al-Arrayed S (2006). Genetic Diseases in Bahrain. In: Tadmouri GO, Taleb Al-Ali M & Al-Khaja N (Eds.) Genetic Disorders in the Arab World: Bahrain. Centre for Arab Genomic Studies, Dubai
5. Al-Hosani H, Salah M, Saade D, Osman H & Al-Zahis J (2003). United Arab Emirates National Newborn Screening Programme: an evaluation 1998-2000. EMHJ -Eastern Mediterranean Health Journal 9(3): 324-332
6. Anonymous (2017). Yoghurt recipe for PKU patients. Retrieved in April, 27, 2017 from https://youtu.be/tGzR953W3N8 AOAC (1990). Official methods of analysis of the association of official analytical chemists. Helrich K (Ed.) Published by the Association of Official Analytical Chemists Inc. Arlington, p, 980.
7. Bahrami M, Ahmadi D, Alizadeh M & Hosseini F (2013.) Physicochemical and sensorial properties of probiotic yogurt as affected by additions of different types of hydrocolloid. Korean Journal for Food Science 33(3): 363-368
8. Barnes H A, Hutton J F & Walters K F R S (1993). An Introduction to Rheology. Elsevıer Science Publichers B.V./ Physical Sciences & Engineering Division, Sara Burgerhartstraat 25, Third impression, AM Amsterdam, The Netherlands.

9. Beshkova D M, Simova E D, Frengova G I, Simov Z I & Adilov E F (1998). Production of amino acids by yogurt bacteria. Biotechnology Progress 14 (6): 963−965
10. Burgard P, Lachmann R H & Walter J (2016). Hyperphenylalaninaemia. In: Saudubray J M, Baumgartner M R & Walter J (Eds.) Inborn Metabolic Diseases, Springer, Berlin, Heidelberg, pp. 251–263
11. Cogan TM (1996) History and taxonomy of starter cultures. In: Congan T M & Accolas J (Eds.) Dairy Starter Cculture. VCH Publishers Inc., New York, pp 1–20
12. CTGA (2021) Phenylketonuria. Centre for Arab Genomic Studies. Retrieved in February, 25, 2021 from https://www.cags.org.ae/en/ctga-details/478/phenylketonuria
13. Cueva O & Aryana K J (2008). Quality Attributes of a Heart Healthy Yogurt. LWT-Food Scien Ceand Technology 41(3): 537-544
14. Çakmakçı S (2012). Gıda Katkı Maddeleri 7. Baskı. Atatürk Üniversitesi Ziraat Fakültesi Ders Yayınları. Atatürk Üniversitesi Ziraat Fakültesi Ofset Tesisi, No: 164, Erzurum, Turkey
15. Dave R I & Shah N P (1996). Evaluation of media for selective enumeration of Streptococcus thermophilus, Lactobacillus delbrueckii ssp. bulgaricus, Lactobacillus acidophilus and bifidobacteria. Journal of Dairy Science 79 (9): 1529- 1536
16. Davis L E, King M K & Schuttz J L (2005). Fundamentals Of Neurologic Disease Demos Medical Publshing, New York
17. Düzgüneş O, Kesici T, Kavuncu O & Gürbüz F (1987). Araştırma ve Deneme Metodları (İstatistik Metodları II) Atatürk Üniversitesi Ziraat Fakültesi Yayınları No: 1021, Ders Kitabı No:295, Ankara
18. El-Metwally A, Al-Ahaidib L Y, Sunqurah A A, Al-Surimi K, Househ M, Alshehri A, Da’ar O B, Abdul Razzak H & AlOdaib A N (2018). The Prevalence of phenylketonuria in Arab Countries, Turkey, and Iran: A Systematic Review. BioMed Research International 2018: 7697210
19. Erdal B & Caferoğlu Z (2018). Fenilketonüride yeni bir tedavi: Pegvaliase. Erciyes Üniversitesi Sağlık Bilimleri Fakültesi Dergisi 5(1): 42-53
20. Goldar P, Givianard M H & Shams A (2016). Effect of ultrafiltered milk permeate and non-dairy creamer powder Cconcentration on low phenylalanine yoghurt’s physicochemical properties during storage. Journal Food Science Technology 53(7): 3053–3059
21. IDF (1986). Determination of nitrogen content (Kjeldahl method) and calculation of crude protein content Standard 20A, International Dairy Federation. Brussels, Belgium.
22. Kavas G & Kınık Ö (2005). İnek sütü ve peynir suyu proteinlerindeki esansiyel amino asitlerin beyin fonksiyonları, psikiyatrik hastalıklar ve süte uygulanan teknolojik parametrelerle ilişkileri. Gıda 30(3): 173-179
23. Koochmeshgi J, Bagheri A & Hosseini-Mazinani S M (2002). Incidence of phenylketonuria in Iran estimated from consanguineous marriages. Journal of Inherited Metabolic Disease 25 (12): 80-81
24. Kosikowski F V (1982). Cheese and Fermented Milk Foods. 2ed published by F.V. Kosikowski and Associates, New York, USA
25. Lee A & Newman J M (2003). Celiac diet: Its impact on quality of life. Journal of the American Dietetic Association 103 (11): 1533-1535
26. Lucey J A (2002). Formation and physical properties of milk protein gels. Journaln of Dairy Science 85: 281-294
27. Macit E & Bakirci İ (2017). Effect of different stablizers on quality characteristics of the set-type yogurt. African Journal of Biotechnology 16(46): 2142-2151 doi: 10.5897/AJB2017.16197
28. MacLeod E L & Ney D M (2010). Nutritional management of phenylketonuria. Annales Nestlé (English ed.) 68: 58–69
29. Meilgaard M C, Civille G V & Carr B T (2006). Sensory Evaluation Techniques, 4rd edn. CRC Press LLC Publishing, Boca Raton, London.
30. Moammar H, Cheriyan G, Mathew R & Al-Sannaa N (2010). Incidence and patterns of inborn errors of metabolism in the Eastern Province of Saudi Arabia, 1983-2008. Annals of Saudi Medicine 30(4): 271–277
31. Mortazavian A M & Sohrabvandi S (2006) Probiotics and food probiotic products: based on dairy probiotic products, Eta Press, Tehran, pp. 31-69 (in Persian)
32. Müslümanoğlu M H, Çine N, Özdemir M, Çilingir O, Başaran N, Durak B, Solak M & Artan S (2014). Fenilketonüri hastalığında prenatal-postnatal tanıda VNTR bağlantısı ve direkt mutasyon analizleri birlikteliğinin avantajları. Kocatepe Tıp Dergisi 5(2): 19-23
33. 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. Journal of Inherited Metabolic Disease 32: 32–39 doi: 10.1007/s10545-008-0952-4
34. Nunes M C, Raymundo A & Sousa I (2006). Gelled vegetable desserts containing pea protein, kapa carrageenan and starch. European Food Research and Technology 222: 622–628
35. Özer E A, İbanoğlu Ş & İbanoğlu E (2008). Fenilketonüri hastalığı ve fenilalanin kısıtlı diyet. In:Türkiye 10. Gıda Kongresi, 21-23 Mayıs, Erzurum, 1139-1140.
36. Parlak Ö (2018). Fenilketonüri hastaları (PKU) ve çölyak hastaları için bisküvi üretimi. Yüksek Lisans Tezi, Bursa Teknik Üniversitesi, Fen Bilimleri Enstitüsü, Bursa
37. Pimentel F B, Alves R C, Costa A S G, Fernandes T J R, Torres D, Almeida M F & Oliveira M B P P (2014). Nutritional composition of low protein and phenylalanine-restricted dishes prepared for phenylketonuria patients. LWT Food Science Technology 57: 283–289
38. PKU F A (2017). Low Proteinli Diet Principles. PKU Family Association. Retrieved in July, 14, 2017 from https://www.pkuaile.com/dusuk-proteinli-diyet-prensipleri
39. Rama Devi A R & Naushad S M (2004). Newborn screening in India. The Indian Journal of Pediatrics 71: 157–160
40. SB (2006). Neonatal Tarama Programı Genelgesi 2006 / 130, Sağlık Bakanlığı, Ankara, Turkey. Retrieved in Fabruary, 25, 2021 from https://www.saglik.gov.tr/TR,11079/neonatal-tarama-programi-genelgesi-2006--130.html
41. Scriver C R & Kaufman S (2001). Hyperphenylalaninemia: phenylalanine hydroxylase deficiency. In: Scriver C R, Beaudet A L, Sly S W & Valle D (Eds.) The Metabolic and Molecular Bases of Inherited Disease. New York: McGraw-Hill, 1667-724.
42. Seçkin Y (2007). Fenilketonürili çocukların psiko-pedegojik sorunları ve çözümleri. In: IX. Uluslararası Katılımlı Beslenme ve Metabolizma Kongresi, 22-25 Ekim, İstanbul, pp 39-43
43. Sellos-Moura M, Glavin F, Lapidus D, Evans K, Carolyn R, Lew C R & Irwin D E (2000). Prevalence, characteristics, and costs of diagnosed homocystinuria, elevated homocysteine, and phenylketonuria in the United States: a retrospective claims-based comparison. BMC Health Services Research 20:183
44. TGK (2003). Fermente sütler tebliği S:24512, Türk Gıda Kodeksi. Tebliğ No: 2001/21 Ankara, Turkey.
45. Thöny B & Blau N (2006). Mutations in the BH4–metabolizing genes GTP cyclohydrolase I, 6pyruvoyl-tetrahydropterin synthase, sepiapterin reductase, carbinolamine-4a-dehydratase, and dihydropteridine reductase. Human Mutation 27: 870-878
46. Üstüner Top F & Küçük Alemdar D (2015). Fenilketonürili çocuğu olan ailelerinin yaşadıkları güçlükler: Niteliksel bir çalışma. Hemşirelikte Eğitim ve Araştırma Dergisi 12 (1): 62-68
47. Van Calcar S C, Mac Load E L, Gleason S T, Etzel M R, Clayton M K, Wolff J A & Ney D M (2009). Improve nutritional management of phenylketonuria by using a diet containing glycomacropeptide with amino acids. The America Journal of Clinical Nutrition 89 (4): 1068–1077
48. Van Wegber A M J, MacDonald A, 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, van Rijn M, Trefz F, Walter J H & van Spronsen F J (2017). The complete European guidelines on phenylketonuria: Diagnosis and treatment. Orphanet Journal of Rare Diseases 12:162
49. Verbeken D, Bael K, Thas O & Dewettinck K (2006). Interactions between κ-carregeenan, milk proteins and modified starch in sterilized dairy desserts. International Dairy Journal 16(2006): 482-488
50. V PKU (2020). Yoghurt Recipe. Vitafrends PKU. Retrieved in December, 25, 2020 from https://www.vitafriendspku.com/tr/tarifler/yogurt/
51. Waisbren S E, Noel K, Fahrbach K, Cella C, Frame D, Dorenbaum A & Levy H (2007). Phenylalanine blood levels and clinical outcomes in phenylketonuria: a systematic literature review and meta-analysis. Moleculer Genetics Metabolism 92(1): 63-70
52. Walter J H, Lee P J & Burgard P (2006). Hyperphenylalaninaemia. In J. Fernandes, J. M. Saudubray, G. van den Berghe, and J. H. Walter (Eds.), Inborn Metabolic Diseases; Diagnosis and Treatment (4th ed., pp. 222-232). Heidelberg, Germany: Springer.
53. Weetch E & Macdonald A (2006). The determination of phenylalanine content of foods suitable for phenylketonuria. Journal of Human Nutrition and Dietetics 19 (3): 229-236