Research progress of diet and nutrition nursing strategy for children with glycogen storage disease typeⅠ
10.3760/cma.j.cn115682-20210421-01757
- VernacularTitle:糖原贮积病Ⅰ型患儿饮食营养护理策略的研究进展
- Author:
Yun JIANG
1
;
Junli YI
;
Genzhen YU
;
Qiao WANG
Author Information
1. 华中科技大学同济医学院附属同济医院儿科，武汉 430030
- Keywords:
Review;
Child;
Diet;
Nutrition;
Glycogen storage disease typeⅠ;
Nursing strategy
- From:
Chinese Journal of Modern Nursing
2021;27(29):4051-4055
- CountryChina
- Language:Chinese
-
Abstract:
Glycogen storage disease type Ⅰ (GSD Ⅰ) is a group of autosomal recessive genetic diseases caused by the lack of glucose-6-phosphatase or its mediated transporter. Due to the disorder of blood glucose metabolism, children will have multiple systemic complications such as lactic acidemia and hepatomegaly, and even die due to severe hypoglycemia. At present, diet and nutrition intervention is still the main treatment and nursing method for this disease. Therefore, this article aims at reviewing the nutritional assessment and dietary nutrition intervention strategies for children with GSDⅠ at home and abroad, so as to provide clinical evidence for clinical medical and nursing staff to take effective diet and nutrition intervention measures, further improve the metabolic control of children with GSDⅠ, reduce the incidence of metabolic complications and improve the quality of life of children.
