Effectiveness of instructional guidelines implementation on mothers' knowledge and practice regarding their children with methylmalonic acidemia
Keywords:
children, instructional guidelines, knowledge, practice, methylmalonic academia, mothersAbstract
Methylmalonic Acidemia (MMA) is a group of inborn errors of metabolism (IEMs), specifically of propionate catabolism characterized by gastrointestinal and neurometabolic manifestations resulting from a deficiency in the function of methyl malonyl-CoA mutase, methylmalonyl-CoA epimerase, and cobalamin metabolism. Aim: To investigate the effectiveness of instructional guidelines implementation on mothers' knowledge and practice regarding their children with methylmalonic academia. Design: A quasi-experimental design was used to achieve the aim of this study. Setting: This study was conducted at the Specialized Genetic Outpatient Clinic at Sohag University Hospital. Subject: A purposive sample of 50 mothers and their children with methylmalonic academia was selected from the previously selected settings. Three tools were used: (1) a structured interviewing questionnaire, (2) Mothers’ knowledge regarding methylmalonic academia, and (3) Mothers’ Reported Practices format regarding methylmalonic academia. Results: The current study results revealed that two-quarters of the mothers had unsatisfactory knowledge about methylmalonic acidemia pre-instructional guidelines implementation compared with the majority of them reported satisfactory total knowledge post-instructional guidelines implementation; As well, mothers' total reported practices were significantly improved post-instructional guidelines implementation.
Downloads
References
Almasi T, Guey LT, Lukacs C, Csetneki K, Voko Z, Zelei T. Systematic literature review and meta-analysis on the epidemiology of methylmalonic acidemia (MMA) with a focus on MMA caused by methylmalonyl-CoA mutase (mut) deficiency. Orphanet. J. Rare Dis. 2019; 14, 84.
Dinchong R. Reducing the psychosocial impact of a false positive newborn screen for inborn errors of metabolism. Faculty of Graduate Studies - FGS - (Electronic Theses & Dissertations) (Public) [24382]. Master of Science, Manitoba Heritage Theses [5726], 2019; https://mspace.lib.umanitoba.ca/handle/1993/34019.
Dixon M, MacDonald A, White F J. Disorders of amino acid metabolism, organic acidaemias and urea cycle disorders. Clinical Paediatric Dietetics, 2020; 513-598.
Dizaji M, Taghdisi M H, Solhi M, Hoseini M, Shafieyan Z, Qorbani M, Rezapoor A. Effects of Educational Intervention Based on PRECEDE Model on Self-Care Behaviors and Control in Patients with Type 2 Diabetes, Journal of Diabetes & Metabolic Disorders; 2014; 13(1), 72.
Evans S, Preston F, Daly A, Ashmore C, Holden C, MacDonald A. Home enteral tube feeding in children with inherited metabolic disorders: a review of long‐term carer knowledge and technique. Journal of Human Nutrition and Dietetics, 2012; 25(6), 520-525.
Forny P, Hörster F, Ballhausen D, Chakrapani A, Chapman K A, Dionisi‐Vici, C, Baumgartner M R. Guidelines for the diagnosis and management of methylmalonic acidaemia and propionic acidaemia: first revision. Journal of Inherited - Metabolic Disease, Wiley O online Library, 2021; https://orcid.org/0000-0003-1877-2976.
Gielen A C, McDonald E M, Gary T L, Bone L R. Using the Precede-PROCEED Model to Apply Health Behavior Theories, Health Behavior and Health Education: Theory, Research, and Practice, 2018; 4:407–29.
Gramer G, Hoffmann G. F. Vitamin B 12 Deficiency in Newborns and their Mothers—Novel Approaches to Early Detection, Treatment, and Prevention of a Global Health Issue. Current Medical Science, 2020; 40(5), 801-809.
Keyfi F, Talebi S, Varasteh A. R. Methylmalonic acidemia diagnosis by laboratory methods. Reports of biochemistry & molecular biology, 2016; 5(1), 1.
Ma M, Wu M, Li Y, Wu D, & Zhang B. Shunt surgery for early-onset severe hydrocephalus in methylmalonic acidemia: report on two cases and review of the literature. Child's Nervous System, 2018; 34(7), 1417-1421.
Maciejewski, M. Quasi-Experimental design. Biostatistics & Epidemiology; 2020; 4 (1): 38-47.
Mazloomy S, Masoudy G, Fallahzadeh H, & Jalili Z. Education based on precede-proceed on quality of life in elderly. Glob J Health Sci; 2014; 6(6):178–84. Doi: 10.5539/gets.v6n6p178.
McLaughlin P M, Hinshaw J, Stringer A Y. Maple syrup urine disease (MSUD): a case with long-term follow-up after liver transplantation. The Clinical Neuropsychologist, 2013; 27(7), 1199-1217.
Ogier de Baulny H, Dionisi-Visi C, Wendel U. (2018) Branched-chain Organic Acidurias/Acidaemias. In Inborn Metabolic Diseases: Diagnosis and Treatment (Saudubray J.M., van den Berge G. & Walter J.H. eds). 5th edition, Springer, Germany, pp. 277-296.
Oruoji MA, Hashemi J, Hazavehei M, Charkazi A, Javaheri J, Moazeni M. The positive impact of an educational intervention program on preventive behaviors to reduce brucellosis in the rural people of Khomeini. Journal of Research Development in Nursing & Midwifery; 2012; 9 (1):51–60.
Pillai N R, Stroup B M, Poliner A, Rossetti L, Rawls B, Shayota B J, Burrage L C. Liver transplantation in propionic and methylmalonic acidemia: A single-center study with literature review, Molecular genetics, and metabolism, 2019; 128(4), 431-443.
Ramadan Z M, Ibrahim I A, Osama K Z. Assessment of Mothers' Knowledge towards Care of Their Children Suffering from Methylmalonic Acidemia (MMA), faculty of nursing, Ain Shams University, 2020; 1-218.
Ramsay J, Morton J, Norris M, Kanungo S. (2018): Organic acid disorders, Ann. Transl. Med; 6, 472.
Ranjbaran S, Dehdari T, Sadeghniiat-Haghighi K, Majdabadi MM. Poor Sleep Quality in Patients after Coronary Artery Bypass Graft Surgery: An Intervention Study Using the PRECEDE Model. J Tehran Heart Cent; 2015; 10(1):1–8.
Shahin M, Mohamed W, Sayed M. Nurses Knowledge and Practices Regarding Enteral Nutrition at the Critical Care Department of Al-Manial University Hospital in Egypt: Impact of a Designed Instructional Program. Journal of American Science, 2012; 8(11), 397-404.
Solhi M, Shabani M, Salehi M. Educational Intervention In Quality of Life of Women-Headed Households in Iran. Medical Journal Islam Repub Iran; 2016; 30:417.
Sutton VR, El-Hattab A W. Inborn Errors of Metabolism, an Issue of Pediatric Clinics of North America, E-Book, 2018; Vol. 65, No. 2.
Tejada-Ortigosa E M, Flores-Rojas K, Moreno-Quintana L, Muñoz-Villanueva M C, Pérez-Navero J L, Gil-Campos M. Health and socio-educational needs of the families and children with rare metabolic diseases: Qualitative study in a tertiary hospital, Anales de Pediatría (English Edition), 2019; 90(1), 42-50.
Zwickler M, Tamaris A, Ariane S. (2018): Metabolic decompensation in methylmalonic aciduria: which biochemical parameters are discriminative? M.D. University Children's Hospital Heidelberg, GERMANY; Journal of Inherited Metabolic Disease.
Published
How to Cite
Issue
Section
Copyright (c) 2021 International journal of health sciences
![Creative Commons License](http://i.creativecommons.org/l/by-nc-nd/4.0/88x31.png)
This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.
Articles published in the International Journal of Health Sciences (IJHS) are available under Creative Commons Attribution Non-Commercial No Derivatives Licence (CC BY-NC-ND 4.0). Authors retain copyright in their work and grant IJHS right of first publication under CC BY-NC-ND 4.0. Users have the right to read, download, copy, distribute, print, search, or link to the full texts of articles in this journal, and to use them for any other lawful purpose.
Articles published in IJHS can be copied, communicated and shared in their published form for non-commercial purposes provided full attribution is given to the author and the journal. Authors are able to enter into separate, additional contractual arrangements for the non-exclusive distribution of the journal's published version of the work (e.g., post it to an institutional repository or publish it in a book), with an acknowledgment of its initial publication in this journal.
This copyright notice applies to articles published in IJHS volumes 4 onwards. Please read about the copyright notices for previous volumes under Journal History.