The Effectiveness of Parenteral Hydroxylcobalamin on Urine Mma in Children with Methylmalonic Acidemia
- سال انتشار: 1402
- محل انتشار: مجله بین المللی کودکان، دوره: 11، شماره: 3
- کد COI اختصاصی: JR_INJPM-11-3_003
- زبان مقاله: انگلیسی
- تعداد مشاهده: 235
نویسندگان
M.D, Fellowship of Pediatric Endocrinology & Metabolism, Department of Pediatrics, School of Medicine, Akbar Hospital, Mashhad University of Medical Sciences, Mashhad, Iran.
Mashhad University of medical sciences
Clinical Research Development Center, Akbar Hospital, Faculty of Medicine, Mashhad University of medical sciences, Mashhad, Iran
nursing and midwifery school, Mashhad University of Medical Sciences, Mashhad, Iran
Clinical Research Development Unit of Akbar Hospital ,Faculty of Medicine , Mashhad University of Medical Sciences, Mashhad, Iran
mashhad university of medicine ,pediatric endocrinology department
چکیده
Background: Methylmalonic acidemia is a rare autosomal recessive disease in which there is a deficiency of intracellular cobalamin. This study aimed to assess the effectiveness of parenteral hydroxylcobalamin in the treatment of children with methylmalonic acidemia. Method: This is a quasi-experimental study without a control group. The participants included under-۱۸-year-old children with confirmed methylmalonic acidemia. There were only ۱۷ patients with the inclusion criteria, all of whom were included in the study. They received ۱mg hydroxylcobalamin injection for ۳ or ۷ days based on their clinical status. Data was gathered by a demographic questionnaire, along with laboratory tests of urine-MMA, and plasma homocysteine, measured before and after the intervention. Data analyses were performed using SPSS v. ۲۶. Results: The samples included ۱۷ patients, most of whom were males (۵۲.۹%). They had various clinical manifestations consisting of hypotonia, seizure, verbal disorders, movement disorders, organomegaly, hematologic disorders, and ophthalmic disorders. The parenteral hydroxylcobalamin had a borderline significant effect on urine-MMA (p=۰.۰۵); this seems to be due to the sample shortage and can become strongly significant with sample increase. Conclusion: The results revealed the effectiveness of parenteral hydroxylcobalamin in MMA patients. However, there is no standard guideline to suggest the perfect dose of it to acquire the optimum result; so it is suggested to conduct more clinical trials or cohort studies to be done.کلیدواژه ها
Hydroxylcobalamin,,, ,،methyl malonic acidemia,,, ,،childrenاطلاعات بیشتر در مورد COI
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