Fenilketonürili Bireylerde Diyet Fenilalanin Toleransının Değerlendirilmesi

Abstract

Lifelong dietary treatment with early diagnosis is the most optimal treatment of phenylketonuria. Low phenylalanine diet is challenging, multifaceted and requires lifelong adherence. There are few number of studies about the factors affecting dietary adherence. Two hundred and forty three patients (114 male, 129 female) aged 10,8 months to 31,9 years (mean age=9.9 years) with phenylketonuria who are older than six months, not breastfed and were followed by Hacettepe University Faculty of Medicine, Department of Pediatrics were recruited. Dietary habits, parental and disease related informations of patients were obtained by a questionnaire using face-to-face interview technique. The energy and nutrient intake of patients were obtained with a parent or self-reported 24-hour food recall. The anthropometric measurements of patients were obtained from their medical records. As a measure of metabolic control, blood phenylalanine levels measured in the last 3 and last 5 years of the patients were obtained and compared with recommended target blood phenylalanine concentrations of Hacettepe University Metabolism Unit. 63,8%, 44,5% and 56% of median blood phenylalanine levels for the past three years of the patients younger than 10 years old, between 11-16 years old and older than 16 years old were above the target range, respectively. The percentage of mean energy, protein, vitamin B12, calcium, zinco and iron intake of patients in relative to the guideline were 79,0%, 99,0%, 92%, 75% 142% and 130%, respectively. Total protein, natural protein, amino acid substitute protein and phenylalanine intake of patients were significantly lower than the recommended dietary intake in the hospital (p<0,01). The percentage of blood phenylalanine levels in the targeted range of patients was shown to be low in all age groups and dietary energy, protein and phenylalanine intake of patients affects dietary adherence. Age is shown to be the most important factor affecting adherence. Potential contributors that leads to poor compliance should be carefully identified and effective strategies should be developed to improve longterm dietary compliance of patients with PKU.