سامانه مدیریت نشریات علمی دانشگاه علوم پزشکی مشهد

Nazari, Afsane, Bagheri, Sepideh, Abbasi Shaye, Zahra, shirdelzade, sara, Pourbadakhshan, Nafiseh, eshraghi, peyman. (1401). The Effectiveness of Parenteral Hydroxylcobalamin on Urine Mma in Children with Methylmalonic Acidemia. , 11(3), 17470-17476. doi: 10.22038/ijp.2023.70401.5182
Afsane Nazari; Sepideh Bagheri; Zahra Abbasi Shaye; sara shirdelzade; Nafiseh Pourbadakhshan; peyman eshraghi. "The Effectiveness of Parenteral Hydroxylcobalamin on Urine Mma in Children with Methylmalonic Acidemia". , 11, 3, 1401, 17470-17476. doi: 10.22038/ijp.2023.70401.5182
Nazari, Afsane, Bagheri, Sepideh, Abbasi Shaye, Zahra, shirdelzade, sara, Pourbadakhshan, Nafiseh, eshraghi, peyman. (1401). 'The Effectiveness of Parenteral Hydroxylcobalamin on Urine Mma in Children with Methylmalonic Acidemia', , 11(3), pp. 17470-17476. doi: 10.22038/ijp.2023.70401.5182
Nazari, Afsane, Bagheri, Sepideh, Abbasi Shaye, Zahra, shirdelzade, sara, Pourbadakhshan, Nafiseh, eshraghi, peyman. The Effectiveness of Parenteral Hydroxylcobalamin on Urine Mma in Children with Methylmalonic Acidemia. , 1401; 11(3): 17470-17476. doi: 10.22038/ijp.2023.70401.5182

The Effectiveness of Parenteral Hydroxylcobalamin on Urine Mma in Children with Methylmalonic Acidemia

Journal of Pediatric Perspectives
مقاله 3، دوره 11، شماره 3، خرداد 2023، صفحه 17470-17476    اصل مقاله (280.69 K)
نوع مقاله: original article
شناسه دیجیتال (DOI): 10.22038/ijp.2023.70401.5182
نویسندگان
Afsane Nazari1؛ Sepideh Bagheri2؛ Zahra Abbasi Shaye3؛ sara shirdelzade4؛ Nafiseh Pourbadakhshan5؛ peyman eshraghi* 6
1M.D, Fellowship of Pediatric Endocrinology & Metabolism, Department of Pediatrics, School of Medicine, Akbar Hospital, Mashhad University of Medical Sciences, Mashhad, Iran.
2Mashhad University of medical sciences
3Clinical Research Development Center, Akbar Hospital, Faculty of Medicine, Mashhad University of medical sciences, Mashhad, Iran
4nursing and midwifery school, Mashhad University of Medical Sciences, Mashhad, Iran
5Clinical Research Development Unit of Akbar Hospital ,Faculty of Medicine , Mashhad University of Medical Sciences, Mashhad, Iran
6mashhad 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-18-year-old children with confirmed methylmalonic acidemia. There were only 17 patients with the inclusion criteria, all of whom were included in the study. They received 1mg hydroxylcobalamin injection for 3 or 7 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. 26.
Results: The samples included 17 patients, most of whom were males (52.9%). 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=0.05); 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.
کلیدواژه‌ها
Hydroxylcobalamin,,؛ ,،methyl malonic acidemia,,؛ ,،children
مراجع
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