Long-term nutritional management of inherited metabolic disorders with hypoglycemia / 中华实用儿科临床杂志

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.