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Journal of Nursing ›› 2021, Vol. 28 ›› Issue (16): 52-57.doi: 10.16460/j.issn1008-9969.2021.16.052

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Formulation of Bestevidence-based Dietary Management Program for Children with Glycogen Storage Disease TypeⅠ

YUAN Lin1, QIU Zheng-qing2a, LI Rong-rong2b, SUN Jing2a, LI Yang3   

  1. 1. Heze Medical College, Heze 274000, China;
    2a. Dept. of Pediatric; 2b. Dept. of Nutritional , Peking Union Medical College Hospital, Beijing 100730, China;
    3. School of Nursing, Peking Union Medical College, Beijing 100144, China
  • Received:2021-04-08 Online:2021-08-25 Published:2021-09-10

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Abstract: Objective To formulate dietary management program for children with glycogen storage disease typeⅠ(GSDⅠ). Methods The framework and the draft of the program was constructed on the basis of literature review and Delphi expert consultations. Results The final program consisted of 7 first-level, 19 second-level and 72 third-level indicators, including the treatment period and the home period. During the treatment period, the first-level items were establishing multidisciplinary team, improving dietary management evaluation, making individualized diet plan and strengthening dietary health education. During the home period, the three first-level items were improving the children’s and their parents’ dietary treatment compliance, paying attention to blood glucose monitoring and adjusting the diet timely. Conclusion Being scientific and reliable, the constructed dietary management program can be promoted in clinical practice.

Key words: glycogen storage disease typeⅠ, dietary management, clinical practice program, Delphi

CLC Number: 

  • R473.72
[1] 中华医学会儿科学分会. 儿科内分泌与代谢性疾病诊疗规范[M]. 北京:人民卫生出版社,2016,333-341.
[2] Kishnani PS, Austin SL, Abdenur JE, et al.Diagnosis and Management of Glycogen Storage Disease Type I: A Practice Guideline of the American College of Medical Genetics and Genomics[J]. Genet Med,2014,16(11):e1. DOI:10.1038/gim.2014.128.
[3] 吴晓珩,杨元立,胡馨予,等. 住院患者输血全流程管理的循证实践[J]. 护理学报,2021,28(8):33-38. DOI:10.16460/j.issn1008-9969.2021.08.033.
[4] Kasapkara CS, Demir GC, Hasanoglu A, et al.Continuous Glucose Monitoring in Children with Glycogen Storage Disease Type I[J]. Eur J Clin Nutr,2014,68(1):101-105. DOI:10.1038/ejcn.2013.186.
[5] 李融融,于康,邱正庆. 北京某综合医院糖原累积症住院患者代谢特点及营养干预现状[J]. 中华临床营养杂志,2017,25(5):272-277. DOI:10.3760/cma.j.issn.1674-635X.2017.05.003.
[6] Bhattacharya K.Dietary Dilemmas in the Management of Glycogen Storage Disease Type I[J]. J Inherit Metab Dis,2011, 34(3):621-629.DOI:10.1007/s10545-011-9322-8.
[7] Chun-Wei LI, Kang YU, Rong-Rong LI, et al.The Medical Nutritional Therapy for Glycogen Storage Diseases:Case Report and Literature Review[J].Acta Nutrimenta Sinica,2014,36(6):594-602.DOI:10.13325/j.cnki.acta.nutr.sin.2014.06.013.
[8] Ross KM, Ferrecchia IA, Dahlberg KR, et al.Dietary Management of the Glycogen Storage Diseases: Evolution of Treatment and Ongoing Controversies[J]. Advances in nutrition,2020,11(2):439-446. DOI:10.1093/advances/nmz092.
[9] Derks TGJ, Van Rijn M.Lipids in Hepatic Glycogen Storage Diseases: Pathophysiology, Monitoring of Dietary Management and Future Directions[J]. J Inherit Metab Dis, 2015,38(3):537-543. DOI:10.1007/s10545-015-9811-2.
[10] Boyer SW, Barclay LJ, Burrage LC.Inherited Metabolic Disorders: Aspects of Chronic Nutrition Management[J]. Nutr Clin Pract, 2015,30(4):502-510.DOI:10.1177/0884533615586201.
[11] Calcar S V.Nutrition Management of Glycogen Storage Disease Type 1[M]. Swizerland: Springer International Publishing,2015:295-319.
[12] Ross KM.General Nutrition Guidelines for Glycogen Storage Disease Type I[DB/OL].[2020-08-13].https://ufhealth.org/sites/default/files/media/GSD/General-Nutrition-Guidelines-for-Glycogen-Storage-Disease-Type-I.pdf.
[13] 邱文娟. 糖原累积病Ⅰ型的营养管理[EB/OL]. (2016-05-31)[2020-08-20].https://www.haodf.com/zhuanjiaguandian/qiuwenjuan_4485162960.htm.
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[10] . [J]. JOURNAL OF NURSING, 2012, 19(2): 44 -45 .