Mothers' Knowledge and Practices Regarding Care of their children Suffering from Maple Syrup Urine Disease. | ||||
| Helwan International Journal for Nursing Research and Practice | ||||
| Article 23, Volume 4, Issue 9, March 2025, Page 320-329 PDF (968.86 K) | ||||
| Document Type: Scientific Research Periodical | ||||
| DOI: 10.21608/hijnrp.2025.324627.1232 | ||||
 View on SCiNiTO | ||||
| Author | ||||
Aisha Ali Ahmed 
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| 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. | ||||
| Keywords | ||||
| Maple Syrup Urine Disease; mothers Knowledge; practices; children | ||||
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