儿童囊性纤维化营养管理——英国皇家布朗普顿医院儿童囊性纤维化照护临床指南(2020)解读

Nutritional management for children with cystic fibrosis:interpretation of clinical guidelines for care of children with cystic fibrosis in Royal Brompton Hospital(2020)

囊性纤维化(CF)是一种单基因遗传病,多脏器受累,营养不良非常常见,与患儿预后及生存期密切相关。CF患者的营养不良与多种因素有关,不同阶段及个体有不同的管理方案,对这些患者进行长期的营养监测与评估非常重要。2020年,英国皇家布朗普顿医院儿童囊性纤维化照护临床指南进行了第8版更新,其中对CF患儿营养管理做了推荐指导,本文对该部分内容进行解读,包括营养管理与评估方法、胰酶替代治疗中的注意事项、营养补充方式选择及如何解决CF儿童喂养困难问题等,意在指导临床医师对CF患者进行早期的营养管理,以期改善患儿预后。

Cystic fibrosis(CF)is a monogenetic disease involving multiple organs.Malnutrition is common in children with CF,and it is closely related to the prognosis and survival of children.Malnutrition is caused by various factors and individualized plans should be made for children with CF at different stages.Besides,it is essentially important to monitor and assess the nutritional status of CF patients in a long term.In 2020,the 8th edition of the clinical guidelines for the care of children with CF in Royal Brompton Hospital was updated,in which the nutritional management of children with CF was recommended and guided.This part of the guidelines was interpreted in the present paper,including nutritional management and evaluation,tips for pancreatic enzyme replacement therapy,types of nutrition supplement and how to solve the feeding difficulties of children with CF.The aim of this paper is to guide early clinical nutritional management of CF patients and improve their prognosis.