Discussion on the principle of protein restriction in the acute stage of hereditary metabolic diseases from the perspective of acrodermatitis with metabolic disorders
10.3760/cma.j.cn101070-20200807-01307
- VernacularTitle:从代谢障碍性肢端皮炎谈遗传代谢性疾病急性期的蛋白限制原则
- Author:
Qiao WANG
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):1541-1545
- CountryChina
- Language:Chinese
-
Abstract:
With the popularization of tandem mass spectrometry and gene detection technology, more and more attention has been paid to hereditary metabolic diseases.The overall incidence rate of hereditary metabolic diseases is 1∶1 000, and the number of patients is huge.The disability and mortality of hereditary metabolic diseases in the acute decompensated period are high.The acute treatment is critical for the prognosis of patients.Limiting the intake of protein is an effective treatment for many patients with amino acid and organic acid hereditary metabolic diseases.However, the lack of standard for protein restriction in the acute decompensation period may lead to " over treatment" in clinical practice and the occurrence of acrodermatitis with hereditary metabolic disorders.In order to standardize and improve the application of dietary therapy in acute treatment, the main points of acute dietary treatment for some common amino acid and organic acid hereditary metabolic diseases were summarized in this paper, and the general protein restriction principle in the acute stage of hereditary metabolic diseases was further explored, so as to provide reference for clinical doctors.
