Ahmed, A. (2025). Mothers' Knowledge and Practices Regarding Care of their children Suffering from Maple Syrup Urine Disease.. Helwan International Journal for Nursing Research and Practice, 4(9), 320-329. doi: 10.21608/hijnrp.2025.324627.1232
Aisha Ali Ahmed. "Mothers' Knowledge and Practices Regarding Care of their children Suffering from Maple Syrup Urine Disease.". Helwan International Journal for Nursing Research and Practice, 4, 9, 2025, 320-329. doi: 10.21608/hijnrp.2025.324627.1232
Ahmed, A. (2025). 'Mothers' Knowledge and Practices Regarding Care of their children Suffering from Maple Syrup Urine Disease.', Helwan International Journal for Nursing Research and Practice, 4(9), pp. 320-329. doi: 10.21608/hijnrp.2025.324627.1232
Ahmed, A. Mothers' Knowledge and Practices Regarding Care of their children Suffering from Maple Syrup Urine Disease.. Helwan International Journal for Nursing Research and Practice, 2025; 4(9): 320-329. doi: 10.21608/hijnrp.2025.324627.1232
Mothers' Knowledge and Practices Regarding Care of their children Suffering from Maple Syrup Urine Disease.
Pediatric department, faculty of nursing, fayoum university, fayoum city.
Abstract
Maple syrup urine disease (MSUD) is a rare inherited autosomal recessive neurometabolic disorder, is brought on by diminished activity of the Branched-chain α-ketoacid dehydrogenase complex (BCKDC), which catalysis the irreversible catabolism of branched-chain amino acids (BCAAs). This disease gets its name because of its burnet sweet odor of the affected infant’s urine. Aim: the current study aimed to assess the mothers' knowledge and practices regarding care for their children suffering from Maple Syrup Urine Disease. Design: A descriptive research design was utilized to fulfill the aim of this study. Setting: The study was carried out at Genetic Out Patient Clinic in Mustafa Hassan Pediatric Hospital affiliated to El-Fayoum University Hospitals, and Abo El Rish (El Monira) hospital affiliated to Cairo University Hospitals. Sample A purposive sample consisted of 71 mothers who had children suffering from Maple Syrup Urine Disease. Tools: consists of two tools were used to conduct this study: Tool I: A structured interview questionnaire sheet to assess mothers' knowledge regarding Maple Syrup Urine Disease. Tool II: Observational checklist to assess mothers' reported practices regarding care of their children with Maple Syrup Urine Disease. Results: The study results showed that, 70.4% of studied mothers had unsatisfactory knowledge about MSUD and 67.6% of them had inadequate reported practices regarding care of their children suffering from MSUD. Conclusion: the current study concluded that the studied mothers had un satisfactory knowledge and practices about maple syrup urine disease. Recommendation: Continuous health education programs to mothers regarding Maple Syrup Urine Disease.
View on SCiNiTO
Top