Abstract：
Nutritional status is paramount in Cystic Fibrosis (CF) and is directly correlated with morbidity and mortality. The first ESPEN-ESPGHAN-ECFS guidelines on nutrition care for infants, children, and adults with CF were published in 2016. An update to these guidelines is presented.
The study was developed by an international multidisciplinary working group in accordance with officially accepted standards. Literature since 2016 was reviewed, PICO questions were discussed and the GRADE system was utilized. Statements were discussed and submitted for on-line voting by the Working Group and by all ESPEN members.
The Working Group updated the nutritional guidelines including assessment and management at all ages. Supplementation of vitamins and pancreatic enzymes remains largely the same. There are expanded chapters on pregnancy, CF-related liver disease, and CF-related diabetes, bone disease, nutritional and mineral supplements, and probiotics. There are new chapters on nutrition with highly effective modulator therapies and nutrition after organ transplantation.
The study was developed by an international multidisciplinary working group in accordance with officially accepted standards. Literature since 2016 was reviewed, PICO questions were discussed and the GRADE system was utilized. Statements were discussed and submitted for on-line voting by the Working Group and by all ESPEN members.
The Working Group updated the nutritional guidelines including assessment and management at all ages. Supplementation of vitamins and pancreatic enzymes remains largely the same. There are expanded chapters on pregnancy, CF-related liver disease, and CF-related diabetes, bone disease, nutritional and mineral supplements, and probiotics. There are new chapters on nutrition with highly effective modulator therapies and nutrition after organ transplantation.
摘要：
背景：营养状况在囊性纤维化（CF）中至关重要，并且与发病率和死亡率直接相关。关于婴儿营养护理的第一个ESPEN-ESPGHAN-ECFS指南，孩子们,和成人CF发表于2016年。介绍了这些指南的更新。
方法：该研究是由国际多学科工作组根据官方认可的标准开发的。对2016年以来的文学进行了回顾，讨论了PICO问题，并利用了GRADE系统。工作组和所有ESPEN成员讨论并提交了声明供在线表决。
结果：工作组更新了营养指南，包括所有年龄段的评估和管理。维生素和胰酶的补充基本上保持不变。有关于怀孕的扩展章节，CF相关性肝病,和CF相关的糖尿病，骨病,营养和矿物质补充剂，和益生菌。有新的章节营养与高效的调制疗法和器官移植后的营养。
方法：该研究是由国际多学科工作组根据官方认可的标准开发的。对2016年以来的文学进行了回顾，讨论了PICO问题，并利用了GRADE系统。工作组和所有ESPEN成员讨论并提交了声明供在线表决。
结果：工作组更新了营养指南，包括所有年龄段的评估和管理。维生素和胰酶的补充基本上保持不变。有关于怀孕的扩展章节，CF相关性肝病,和CF相关的糖尿病，骨病,营养和矿物质补充剂，和益生菌。有新的章节营养与高效的调制疗法和器官移植后的营养。
