Volume 8, Issue 2, March 2020, Page: 16-23
Development of a Diet Management Scale for Pediatric Patients with Citrin Deficiency
Ling Yan, Department of Nursing, The First Affiliated Hospital of Jinan University, Guangzhou, China
Yuanzong Song, Department of Pediatrics, The First Affiliated Hospital of Jinan University, Guangzhou, China
Meng Zhang, Department of Nursing, The First Affiliated Hospital of Jinan University, Guangzhou, China
Jianwu Qiu, Department of Pediatrics, The First Affiliated Hospital of Jinan University, Guangzhou, China
Chong Jiang, Department of Stomatologist, Nanfang Hospital, Southern Medical University, Guangzhou, China
Qingran Lin, Department of Nursing, The First Affiliated Hospital of Jinan University, Guangzhou, China
Received: Mar. 15, 2020;       Accepted: Mar. 31, 2020;       Published: Apr. 23, 2020
DOI: 10.11648/j.ejpm.20200802.12      View  324      Downloads  120
Objective: This study aimed to develop a scale to assess the caregiversability to manage the dietary needs of pediatric patients with citrin deficiency (CD). Methods: 24 experts from 11 hospitals in mainland China participated in the Delphi research. Item analysis was performed on 61 caregivers of CD patients who were enrolled in the testing of the draft scale, and 153 caregivers attended the validity and reliability testing. The initial scale was made according to the suggestions by the 24 experts. Retest reliability method and Cronbach’s α coefficient method were used to evaluate the external and internal reliability of the scale, respectively; the scale validity was evaluated by content validity and construct validity. Results: A consensus was reached by 24 experts after two rounds of investigation, and a preliminary draft of the scale was formed consisting of three dimensions: Special dietary knowledge, Healthy dietary attitude and Dietary behavior. The expert authority coefficient was 0.887, and the Kendall coefficient was 0.201. The final scale Cronbach's alpha coefficient (α = 0.713) showed good internal consistency and balance stability. The retest reliability coefficient of the scale was 0.975, indicating good external stability. The total content validity index (S-CVI) was 0.981, and the content validity index (I-CVI) of each item varied between 0.913 and 1.0. On confirmatory factor analysis, the indicators CMIN/DF, RESAE, PNFI, NFI, TLI, IFI and CFI for the construct validity of the scale were 1.191, 0.053, 0.628, 0.796, 0.940, 0.954, and 0.951, respectively. Conclusion: The scale developed in this paper exhibited promising reliability and validity for the clinicians to evaluate the dietary management ability of the caregivers of pediatric CD patients.
Scale, Citrin Deficiency, Diet Manage, Caregiver
To cite this article
Ling Yan, Yuanzong Song, Meng Zhang, Jianwu Qiu, Chong Jiang, Qingran Lin, Development of a Diet Management Scale for Pediatric Patients with Citrin Deficiency, European Journal of Preventive Medicine. Vol. 8, No. 2, 2020, pp. 16-23. doi: 10.11648/j.ejpm.20200802.12
Copyright © 2020 Authors retain the copyright of this article.
This article is an open access article distributed under the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0/) which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Kobayashi K, Sinasac D, Iijima M, et al. The gene mutated in adult-onset type II citrullinaemia encodes a putative mitochondrial carrier protein [J]. Nature Genetics, 1999, 22 (2): 159-163.
Song Y Z, Deng M, Chen F P, et al. Genotypic and phenotypic features of citrin deficiency: Five-year experience in a Chinese pediatric center [J]. International Journal of Molecular Medicine, 2011, 28 (1): 33-40.
Fukushima K, Yazaki M, Nakamura M, et al. Conventional Diet Therapy for Hyperammonemia is Risky in the Treatment of Hepatic Encephalopathy Associated with Citrin Deficiency [J]. Internal Medicine, 2010, 49 (3): 243-247.
Imamura Y, Kobayashi K, Shibatou T, et al. Effectiveness of carbohydrate-restricted diet and arginine granules therapy for adult-onset type II citrullinemia: a case report of siblings showing homozygous SLC25A13 mutation with and without the disease [J]. Hepatology Research, 2003, 26 (1): 68-72.
Kimura N, Kubo N, Narumi S et al. Liver Transplantation Versus Conservative Treatment for Adult-Onset Type II Citrullinemia: Our Experience and a Review of the Literature [J]. Transplantation Proceedings, 2013, 45 (9): 3432-3437.
Hayasaka K, Numakura C, Toyota K, et al. Medium-chain triglyceride supplementation under a low-carbohydrate formula is a promising therapy for adult-onset type II citrullinemia [J]. Molecular Genetics and Metabolism Reports, 2014, 1: 42-50.
Saheki T, Kobayashi K, Terashi M, et al. Reduced carbohydrate intake in citrin-deficient subjects [J]. Journal of Inherited Metabolic Disease, 2008, 31 (3): 386-394.
Hayasaka K, Numakura C, Yamka M, et al. Medium-chain triglycerides supplement therapy with a low-carbohydrate formula can supply energy and enhance ammonia detoxification in the hepatocytes of patients with adult–onset type II citrullinemia [J]. Journal of Inherited Metabolic Disease, 2018, 41 (5): 777-784.
Numakura C, Tamiya G, Ueki M, et al. Growth impairment in individuals with citrin deficiency [J]. Journal of Inherited Metabolic Disease, 2019, 42 (3): 501-508.
Ge G. Development and evaluation of pre-diabetes self-management scale, [D]. Jinan University, 2016.
Huang M, Gu Y, Zhang Y X, et al. Development and Evaluation of Evidence and Validity of Evidence-based Nursing Practice Readiness Assessment Scale [J]. Chinese Journal of Evidence-based Pediatr, 2017, 12 (2): 121-125.
Rong Y M, Zhang Y X, Lou J H, et al. Development of patient care and family-centered nursing process scale and its reliability and validity evaluation [J]. Chinese Journal of Evidence-based Pediatr, 2015, 10 (2): 136-141.
Wu M L. Questionnaire statistical analysis practice-SPSS operation and application [M]. Chongqing University Press, 2010: 159-160.
Song Y Z, Zhang Z H, Lin W X, et al. SLC25A13 gene analysis in citrin deficiency: sixteen novel mutations in East Asian patients, and the mutation distribution in a large pediatric cohort in China [J]. PloS ONE, 2013, 8 (9): e74544.
Zhang Z H, Yang ZG, Chen F P, et al. Screening for five prevalent mutations of SLC25A13 gene in Guangdong, China: a molecular epidemiologic survey of citrin deficiency [J]. The Tohoku Journal of Experimental Medicine, 2014, 233 (4): 275-281.
Xu J, Gao M. Analysis of SLC25A13 gene mutations in five infants with neonatal intrahepatic cholestasis caused by citrin deficiency [J]. Chinese journal of medical genetics, 2018, 35 (1): 34-38.
Zeng G. Modern epidemiological methods and application [M]. Beijing Medical University/China Union Medical University Joint Press, 1994: 250-270.
Browse journals by subject