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Background: Maple syrup disease (MSD) is an inherited metabolic disease that has the capacity to kill or cause severe neurological damage to children at early years of life. The aim of this study was to evaluate the effect of Educational Program on nurses' management of children with Maple Syrup Urine Disease during acute intermittent late-onset. A quasi experimental design was used to conduct this study. Settings: The study was carried out in the Pediatric unit and hereditary unit at Ain Shams Specialized Hospital for children affiliated to Ain shams University. Sample: A convenient sample of all nurses working in pediatric and hereditary units (80 bedside male and female nurses) from the beginning of February 2016 to the end of July 2016. Two tools were utilized to collect data for this study; A structured Interviewing questionnaire sheet, and observational checklists regarding nursing care provided for children with MSUD. Results: nurses' knowledge regarding MSUD was unsatisfactory on pre – program phase, while there is a significant statistical difference pre and post program implementation. There is a positive relation between nurses' knowledge, practice and personal data pre and post program implementation. Conclusion: There is an improvement in nurses' practice between pre and post program implementation regarding management of children with MSUD. The study revealed that the program had positive effect on the nurses' knowledge and practice towards management of children with MSUD. Recommendations: The study recommended that continuous education and training sessions about management of children with MSUD should be provided to upgrade nurses' knowledge and improve their practice level. Availability and accessibility of printed universal guidelines and standard precautions regarding MSUD.

Educational Program for Nurses Regarding Management of Children with Maple Syrup Urine Disease during Acute Intermittent Late- Onset