Long-term nutritional management of inherited metabolic disorders with hypoglycemia
10.3760/cma.j.cn101070-20200828-01433
- VernacularTitle:伴有低血糖的先天遗传代谢性疾病长期营养管理
- Author:
Chang SU
1
;
Chunxiu GONG
Author Information
1. 国家儿童医学中心，首都医科大学附属北京儿童医院内分泌遗传代谢科，北京 100045
- From:
Chinese Journal of Applied Clinical Pediatrics
2020;35(20):1549-1552
- CountryChina
- Language:Chinese
-
Abstract:
The development of newborn screening has led to the diagnosis of an expanding population of patients with inherited metabolic disorders.There are many types of inherited metabolic diseases, some of which are accompanied by episodic or persistent hypoglycemia.Severe episodes of hypoglycemia may aggravate metabolic crisis, as well as brain and vision damage.Nutritional management is the main treatment for inherited metabolic diseases.Correct diet management can reduce or even avoid hypoglycemia attacks while maintaining normal growth and development, inhibit the generation of harmful metabolites, and improve the quality of life of patients.Patients with inherited metabolic disorders complicated by hypoglycemia should adhere to nutrition management throughout their entire life.In this article, the current status of chronic nutritional management of inherited metabolic diseases with hypoglycemia was reviewed and related recommendations were made.
