Dietary therapy for inherited metabolic diseases
10.3760/cma.j.cn101070-20201221-01922
- VernacularTitle:遗传代谢病的饮食治疗
- Author:
Lifang FENG
1
;
Chunxiu GONG
;
Lianshu HAN
Author Information
1. 华中科技大学同济医学院附属武汉儿童医院遗传代谢内分泌科，武汉 430016
- Keywords:
Inherited metabolic disease;
Dietary therapy;
Disorders of amino acid and peptide metabolism;
Disorders of lipid and lipoprotein metabolism;
Disorders of
- From:
Chinese Journal of Applied Clinical Pediatrics
2022;37(11):870-874
- CountryChina
- Language:Chinese
-
Abstract:
The developments of mass spectrometry and gene detection technology and the introduction of newborn screening have led to an expanding population of patients with inherited metabolic diseases.Therapies of inherited metabolic diseases have attracted much attention.The basic principles of management in these diseases are to limite the consumption of nutrients that produce toxic products, supplement deficient substances, and increase excretion of toxic metabolites.Dietary therapy is one of the major treatments for many inherited metabolic disorders, with the starting point of limiting the intake of substrates for metabolic disorders and supplementing products of insufficient synthesis or alternative energy sources to bypass the defective pathway in order to maintain normal growth and development.With more and more special medical formula nutritional foods being put into production and use, dietary therapy become accessible and compliant.With the effective dietary therapy, many patients get clinical symptom controlled, and their quality of life has been improved.This article mainly elaborates the common inherited metabolic diseases dietary therapy.
