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. 2024 Feb;43(2):413-445.
doi: 10.1016/j.clnu.2023.12.017. Epub 2023 Dec 27.

ESPEN-ESPGHAN-ECFS guideline on nutrition care for cystic fibrosis

Michael Wilschanski  1 , Anne Munck  2 , Estefania Carrion  3 , Marco Cipolli  4 , Sarah Collins  5 , Carla Colombo  6 , Dimitri Declercq  7 , Elpis Hatziagorou  8 , Jessie Hulst  9 , Daina Kalnins  10 , Christina N Katsagoni  11 , Jochen G Mainz  12 , Carmen Ribes-Koninckx  13 , Chris Smith  14 , Thomas Smith  15 , Stephanie Van Biervliet  16 , Michael Chourdakis  17
Affiliations

ESPEN-ESPGHAN-ECFS guideline on nutrition care for cystic fibrosis

Michael Wilschanski et al. Clin Nutr. 2024 Feb.

Abstract

Background: 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.

Methods: 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.

Results: 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.

Keywords: Cystic fibrosis; Guideline update; Nutrition.

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Conflict of interest statement

Conflict of interest The expert members of the working group were accredited by the ESPEN Guidelines Group, the ESPEN Education and Clinical Practice Committee, and the ESPEN executive. All expert members have declared their individual conflicts of interest according to the rules of the International Committee of Medical Journal Editors (ICMJE). If potential conflicts were indicated, they were reviewed by the ESPEN guideline officers and, in cases of doubts, by the ESPEN executive. None of the expert panel had to be excluded from the working group or from co-authorship because of serious conflicts. The conflict of interest forms are stored at the ESPEN guideline office and can be reviewed with legitimate interest upon request to the ESPEN executive.