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Phenylketonuria also identified as (phenylalanine hydroxylase deficiency) is an autosomal falling syndrome of phenylalanine metabolic rate, in which particularly high phenylalanine focusses cause mind dysfunction. If crude, these mind dysfunction consequences in severe knowledgeable disability, epilepsy and behavioral difficulties. Purpose: To Assess Parents' Performance according to their assessment of nutritional management; to Identify the association between parents’ performance about nutritional management and their demographic variables of children (age, gender, and parents’ Socioeconomic Status). Methods: A study was conducted in the following Ibn Al-Baladi for Maternity and Pediatric Hospital (Metabolic disorders and neonatal screening consultants). A non-experimental design; Data was collected after the approval of parents to involve themselves and their children in the study. data are analyzed using Statistical Data Analysis. an instrument was built for parents' nutritional management for their children diagnosed with phenylketonuria to assess the parents' performance. Results: Outcomes presented that 85.7% of parents are showing moderate performance about of nutritional management for children with phenylketonuria during pre-test time, while they show very good level of performance during the post-test time 1 and post-test time 2 (88.6%). Conclusion: Overall Assessment of Parents’ Performance showing moderate performance about nutritional management, their performance level is increased to good during the post-test time.
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