Problem-Solving Skills in Children with Early Treated Phenylketonuria مقاله

Objectives:This study aimed to investigate the level of problem-solving skills in 1-4 year-old children with early treated phenylketonuria (ETPKU). Method:This analytic, cross-sectional study was conducted on 70 1-4 year-old children referred to phenylketonuria (PKU) clinics that had been diagnosed on screening at birth and had been treated. The measurements were a demographic questionnaire, Ages & Stages Questionnaire (ASQ) and clinical assessment by a pediatrician. One hundred children were also selected randomly from Tehran’s kindergartens as the control group, who also completed the above questionnaires. Data were analyzed by SPSS 19.0 software. Results:The mean age of the children was 29.71 months in the control group and 28.51 months in the case group. There were no significant differences between the two groups regarding the type of delivery and the current and birth heights, weights and headcircumferences. There was a significant difference between the two groups regarding problem-solving developmental skills (P <0.000). The level of problem-solving developmental skills in the children with ETPKU was lower than normal children. Conclusion:It seems that Iranian children with ETPKU, regardless of being on a diet, have lower problem-solving skills. It is recommended to revise their diet and treatment and also to plan programs for early detection, and to carry out interventions for developmental delays in these children.

Iranian Rehabilitation Journal, Vol. 11, No. 18, October 2013 Original Article Problem-Solving Skills in Children with Early Treated Phenylketonuria Firoozeh Sajedi, MD. University of Social Welfare and Rehabilitation Sciences, Tehran, Iran Objectives: This study aimed to investigate the level of problem-solving skills in 1-4 year-old children with early treated phenylketonuria (ETPKU). Method: This analytic, cross-sectional study was conducted on 70 1-4 year-old children referred to phenylketonuria (PKU) clinics that had been diagnosed on screening at birth and had been treated. Keywords: Early treated Phenylketonuria (ETPKU), problem-solving skills, children Submitted: 12 July 2013 Accepted: 11 September 2013 Introduction Phenylketonuria is the most prevalent metabolic disorder that is inherited as an autosomal recessive condition. The aim of this study was to determine the level of problem-solving developmental skills in 1-4 year-old children with ETPKU, and if need be, to recommend early intervention and rehabilitation for these children. The inclusion criteria were: neonatal diagnosis of PKU, early and continuous treatment with a phenylalanine-restricted diet (The mean blood Phe level during the recent 6 months was 2-6 mg/dl or 120- 360 mmol/l), and 1 to 4 years of age. The demographic questionnaire included familial and medical histories, including age, sex, height, weight and head circumference at birth and present, consanguinity of the parents and recorded phenylalanine levels. The mean score of developmental problem solving skills in normal and PKU children were 51. 000 Discussion This study showed that the level of developmental problem-solving skills in ETPKU is lower than in normal children.