Abstract

Objective: The aim of this study was to determine the effect of maternal feeding behavior on nutrition status and behaviors of children with phenylketonuria (PKU).

Material and Methods: The PKU Family Association’s 2-11 year-old children and their mothers were administered face to face questionnaire to determine the sociodemographic characteristics of the family, mothers’ feeding attitudes of their children and anthropometric measurements of the children. One-day food consumption records were taken retrospectively, daily energy, macro and micro nutrients were calculated by the Nutrition Information System computer program and the level of meeting daily requirements was determined. Children’s anthropometric evaluation was done with WHO AnthroPlus Program, weight for age z-score (WAZ), height for age z-score (HAZ) and BMI for age z-score (BAZ) were calculated.

Results: It was found that the mean WAZ value of the children was 0.1 ± 0.9, the HAZ value was -0.3 ± 1.7 and the BAZ value was 0.4 ± 1.8 and the feeding behavior of the mother affected the anthropometric measures of the child (p<0.05). While energy requirements were met by 77.7%, protein, vitamin B6, B12 and C intake was above the recommendations and folate intake was below the recommendations. It was detected that the feeding behavior of the mother when child refused food intake, did not affect the child’s blood phenylalanine level and macro-micro nutrient intake (p>0.05). The majority of the children (86.7%) were found to be compatible with their diet and their mothers were found to higher age (p = 0.03). It was determined that the majority of mothers (80.0%) did not give up when they refused to feed and that feeding (26.7%) after feeding them for a while and trying to feed them into games (23.3%).

Conclusion: Feeding attitudes of mothers affect the nutritional status of children. In order to increase compliance with the lifelong dietary treatment of children with PKU, supportive nutrition education for parents should be planned and provided part of the treatment.

Keywords: Phenylketonuria,, Metabolic disorders, Eating behavior,, Nutritional status,

References

  1. Türkiye İstatistik Kurumu Aile Yapısı Araştırması (TÜİK). Erişim tarihi: 16 Eylül 2019. E erişim https://ailevecalisma.gov.tr
  2. Tokatlı A. Doğuştan metabolik hastalıklara tanısal yaklaşım. Güncel Pediatri 2006; 4:133-8.
  3. Tolunay İ, Yıldızdaş RD, Horoz ÖÖ, Petmezci E, Ekinci F, Mungan N. Doğuştan metabolik hastalıkların tedavisinde ekstrakorporeal yöntemler. Arşiv Kaynak Tarama Dergisi 2015;4:509-19.
  4. Köksal G, Özel Gökmen H. Çocuk Hastalıklarında Beslenme Tedavisi, Doğuştan Metabolizma Hastalıkları. 1. Baskı. Ankara: Hatiboğlu Basım ve Yayım San, Tic, Ltd, Şti, 2015:359-547.
  5. Blau N, Spronsen FJ, Levy HL. Phenylketonuria. Lancet 2010;376:1417-27.
  6. Ahring K, Be ́langer-Quintana A, Dokoupil K. Dietary management practices in phenylketonuria across European centres. Clin Nutr 2009;28:231-6.
  7. Loeber JG. Neonatal screening in Europe; the situation in 2004. J Inherit Metab Dis 2007;30:430–8.
  8. Özbaş S. Türkiye’de yenidoğan tarama programı. Uluslararası Katılımlı IX. Metabolik Hastalıklar ve Beslenme Kongre Kitabı. Uluslararası Katılımlı IX. Metabolik Hastalıklar ve Beslenme Kongresi; 2011; İzmir, Türkiye; 2011. p. 4.
  9. Büyükgebiz B, Öztürk Y. Doğumsal metabolik hastalıklarda beslenme. Klin Cocuk Forum 2002;2:38-44.
  10. Erol S, Işık DU, Çelik İH, Baş AY, Demirel N. Maternal fenilketonüri sendromuna bağlı çoklu konjenital anomali gelişen iki olgu. Türkiye Çocuk Hast Derg 2016;11:286-8.
  11. Al Hafid N, Christodoulou J. Phenylketonuria: a review of current and future treatments. Transl Pediatr 2015;4:304-17.
  12. Çevik A, Ertaş N. Fenilketonüri hastalığı ve hastalığa uygun gıda üretimi. Bozok Tıp Dergisi 2019;10:256-63.
  13. Köksal G, Özel Gökmen H. Metabolik hastalıklarda beslenme 1st ed. Ankara: Sağlık bakanlığı yayınları, 2012:1-37. E erişim: https://sbu.saglik.gov.tr/Ekutuphane/kitaplar/t56.pdf
  14. Çoker M. Metabolik hastalıklarda beslenme. İçinde: Selimoğlu MA (edt) Sağlıkta ve Hastalıkta Çocuk Beslenmesi. 1. Baskı. İstanbul: Akademi Yayınevi, 2014:403-416.
  15. Crone MR, Van Spronsen FJ, Oudshoorn K, Bekhof J, Van Rjin G, Verkerk PH. Behavioural factors related to metabolic control in patients with phenylketonuria. J Inherit Metab Dis 2005;28:627-37.
  16. Evans S, Daly A, Chahal S, MacDonald J, MacDonald A. Food acceptance and neophobia in children with phenylketonuria: a prospective controlled study. J Hum Nutr Diet 2016;29: 427-33.
  17. Top FÜ, Alemdar DK. Fenilketonürili çocuğu olan ailelerinin yaşadıkları güçlükler: Niteliksel bir çalışma. Hemşirelikte Eğitim ve Araştırma Dergisi 2015; 12: 62-8.
  18. Besler HT, Rakıcıoğlu N, Ayaz A, Büyüktuncer Demirel Z, Gökmen Özel H, Samur F ve ark. Türkiye’ye özgü besin ve beslenme rehberi. Ankara: Merdiven Reklam Tanıtım, 2015:96.
  19. World Health Organization WHO AnthroPlus for personal computers Manual: Software for assessing growth of the world’s children and adolescents. Erişim tarihi: 16 Eylül 2019. Available from: https://www.who.int/
  20. Türkiye İstatistik Kurumu (TÜİK) Sağlık Araştırması (Health Survey) 2010. Erişim tarihi: 16 Eylül 2019. E erişim: http://www.tuik. gov.tr/
  21. Acosta PB, Yannicelli S, Singh R, Mofidi S, Steiner R, DeVincentis E, ve ark. Nutrient intakes and physical growth of children with phenylketonuria undergoing nutrition therapy. J Am Diet Assoc 2003;103:1167-73.
  22. Giovannini M, Verduci E, Salvatici E, Fiori L. Phenilketonuria: Dietary and therapeutic challenges. J Inherit Metab Dis 2007;30:145-52.
  23. Okano Y, Hattori T, Fujimoto H, Noi K, Okamoto M, Watanabe T, ve ark. Nutritional status of patients with phenylketonuria in Japan. Mol Genet Metab Rep 2016;8:103-10.
  24. MacDonald A, Davies P, Daly A, Hopkins V, Hall SK, Asplin D, ve ark. Does maternal knowledge and parent education affect blood phenylalanine control in phenylketonuria? J Hum Nutr Diet 2008;21:351-8

How to cite

1.
Aktaç Ş, Öğren G, Fereli S, Karğın D, İçen H. The Effect of Maternal Feeding Behaviours on Eating Attitudes and Behaviours of Children with Phenylketonuria. Turk J Pediatr Dis [Internet]. 2021 May 25 [cited 2025 Aug. 23];15(3):174-80. Available from: https://turkjpediatrdis.org/article/view/783