Fenilketonürili Çocuklarda Diyet Tedavisi ve Obezite Sıklığı

Abstract

Fenilketonüri (FKU), amino asit metabolizmasının en sık görülen, doğuştan gelen, otomozal resesif geçen ve kan fenilalanin seviyesini normal sınırlarda tutmak için düşük fenilalanin diyetinin uygulandığı bir hastalıktır. FKU hastaları obeziteye yatkınlık yaratabilecek karbonhidratlardan ve yağlardan zengin bir diyet tüketme eğilimindedir. Ayrıca FKU’lu bireylerin yeme davranışlarının, düşük proteinli özel ürün/besin içeriğinin, artan protein ikamesi kullanımlarının, egzersiz eksikliğinin, FKU tiplerinin, kadın cinsiyetin ve adölesan dönemin obezite etiyolojisinde yer alabileceği bildirilmiştir. Bazı eski çalışmalar FKU’lu hastalarda obezitenin artışını gösterirken bazı çalışmaların sonuçları tutarsızdır. Tedavi stratejilerindeki farklılıklar, kan fenilalanin konsantrasyonları için hedef aralıklar ve FKU'nun şiddeti, sonuçta aşırı kilo gelişiminin seyrini modüle eden beslenme alımlarını ve diyet deneyimlerini değiştirebilir. Bu sebeple bu derlemede diyet tedavisi ve ilişkili diyet faktörlerinin obezite sıklığına etkisini değerlendirmek amaçlanmıştır. Bununla birlikte FKU tedavisinde, FKU’lu bireylerde obezitenin önlenmesi için beslenme ekibinin beslenme, yeme davranışı ve egzersizin önemine dikkat etmesi gereklidir.

Keywords

• Fenilketonüri
• Fenilalanin
• Diyet Terapisi
• Beslenme Durumu
• Obezite.

Diet Therapy and Obesity Prevalence in Children with Phenylketonuria

Abstract

Phenylketonuria (PKU) is the most common congenital disorder of amino acid metabolism, inherited in an autosomal recessive manner, and requires a low-phenylalanine diet to maintain blood phenylalanine levels within normal limits. Therefore, PKU patients tend to consume a diet rich in carbohydrates and fats, which can predispose to obesity. It has also been reported that eating behaviors of individuals with FKU, low-protein specialty product content, increased use of protein substitutes, lack of exercise, types of PKU, female gender and adolescence may be involved in the etiology of obesity. While some older studies have shown an increase in obesity in patients with PKU, the results of other studies are inconsistent. Differences in treatment strategies, target ranges for blood phenylalanine concentrations and severity of PKU may alter dietary intakes and dietary experiences that ultimately modulate the course of overweight development. This review therefore aims to assess the impact of dietary treatment and associated dietary factors on the prevalence of obesity. However, in the treatment of PKU, the nutrition team should pay attention to the importance of nutrition, eating behavior and exercise to prevent obesity in individuals with PKU.

Keywords

• Phenylketonuria
• Phenylalanine
• Diet Therapy
• Nutritional Status
• Obesity.

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