Fenilketonürili Hastalarda Büyümenin Değerlendirilmesi

Abstract

Background: The aim of this study was to assess the effects of low-protein and low phenylalanin diet to height and weight on patients with phenyketonuriaand to compare sensitivity of two different growth charts in the assessment of growth in PKU children. Methods: 485 patients with the diagnosis of phenylketonuria followed up between years 1995 and 2012 were included in the study. Date of last visit, age, sex, date of diagnosis, blood phenylalanine levels at diagnosis, height and weight at last visit, follow up blood phenylalanine levels, dietary protein and phenylalanine intakes per kg of body weight, energy intake, results of the pshychometric evaluation of the patients were collected retrospectively. WHO body weight for age percentiles and standard deviation reference values, CDC weight for age percentiles, WHO height/length for age percentiles and standard deviation reference values, CDC height/length for age percentiles and standard deviation reference values, weight for length, body mass index, WHO body mass index for age percentiles and standard deviation reference values, CDC body mass index for age percentiles were noted. Weight and height data of parents of short statured and/or overweight patients, were also collected and target heights of the patients were calculated. Obesity, growth retardation, short stature prevelances were estimated. Growth data obtained with CDC-2000 and WHO growth charts were compared. Results: 9 patients (2.1%) were underweight, 63 patients (14.7%) were overweight and 52 patients (12.1%) were obese according to CDC-2000 growth charts. According to WHO 2006-2007 growth charts 114 patients (23.5%) were overweight, 49 patients (10.1%) were obese and 4 patients (0.8%) were underweight. The prevalence of overweight was significantly high in the 5-11 year age group. It was found that WHO growth charts detect overweight patients betterthan CDC growth charts. It was found that the patients with phenylketonuria had significantly higher prevelance of overweight.65 patients (13.4%) were short statured according to WHO and CDC growth charts.There was similarity according to kappa statistical evaluation (Kappa value 0.82) between the two growth charts. It was found that the frequency of short stature was significantly higher in the 5-15 year age group of patients with phenylketonuriathan in the Turkish children. Conclusions:Patients with phenylketonuria are predisposed to be overweight and short. WHO growth charts detect overweight patients better than CDC growth charts. Further studies are needed to find out that what specific components of the diet are playing role in the causation of such changes in the nutritional status of the phenylketonuria children.