Determination of malnutrition status of preschool children with classical phenylketonuria

Bakırel, N.A., Determination of malnutrition status of preschool children with classical phenylketonuria. Gazi University Institute of Educational Sciences. Msc Thesis in Department of Household Economy and Nutrition Education, Ankara, 2007. The aim of this study to evaluate nutrient intakes, physical growth and malnutrition status of preschool children with clasical phenylketonuria undergoing nutritional management. This study was planned and conducted on 70 children with phenylketonuria between the age of 1-7 treated at the Hacettepe University İhsan Doğramacı Children?s Hospital?s Department of Metabolism and Dietetics. The general information, statusof the illness and nutrition habits of the patients were obtained with face to face contact and using a questionnaire. The nutritional status of phenylketonuric children was assessed by a 24 hour diet consumption recall with antropometric measurements ( weight, height, head circumference, mid upper arm circumference) and biochemical parameters. Malnutrition status of the children was assessed by the measurement of ?height for age?, ?weight for height? and ?weight for age?. Age and gender specific ?National Center for Health Statistics/Center for Diseases Control/World Health Organization (NCHS/CDC/WHO)? reference categories were used. ?Height for ages? reflects achieved linear growth and its deficits indicate long term, chronic inadequacies of nutrition. We evaluated the chronic malnutrition status (?-2 SD, <5.percentil) with ?height for age? (shortness) of children aged 1-3 years for according to the z-scores and percentiles that they are found % 18.2 and % 22.7; for 4-6 ages % 30.8 and % 46.2 respectively. And also we evaluated the acute malnutrition status (?-2 SD, <5.percentil) with ?weight for ages? (underweight) according to the Z-scores and percentiles that thay are found % 20.5 and % 27.3; for 4-6 ages %15.4 and %26.9 respectively. The intake of phenylalanine, protein and energy of the children has found lower than the recommended daily amounts (p<0.05). The intakes of vitamine A, E, B1, B2, B6, C ve K are found significantly higher than the dietary reference intakes of the children aged 1-3 years (p<0.05). However, the niasin (p>0.05) , vitamine D (p>0.05), the vitamine B12 (p<0.05) and fiber (p<0.05) are taken lower than the dietary reference intakes (DRI) in aged 1-3 years. The intakes of vitamine A (p<0.05), vitamine E (p<0.05), vitamine B6 (p<0.05), vitamine C (p<0.05), vitamine K (p<0.05) and vitamin B2 (p>0.05) are taken higher than dietary reference intakes the of the children aged 4-6 years. However, the niasin (p<0.05), vitamine B12 (p<0.05), vitamine D (p<0.05) and vitamine D are taken lower than the dietary reference intakes in aged 4-6 years. In order to improve their dietary habits, and preserve their health in the advanced period of their lives, we concluded that children and their family education on a sufficient and balanced diet would be benefical. development and growth of the children with PKU who have restricted diet treatment should be strictly followed and their biochemical assessments should be done and balanced diet conditions should be provided.Key words: Phenylketonuria, malnutrition, phenylalanine.