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

Michael Wilschanski; Anne Munck; Estefania Carrion; Marco Cipolli; Sarah Collins; Carla Colombo; Dimitri Declercq; Elpis Hatziagorou; Jessie Hulst; Daina Kalnins; Christina N Katsagoni; Jochen G Mainz; Carmen Ribes-Koninckx; Chris Smith; Thomas Smith; Stephanie Van Biervliet; Michael Chourdakis

doi:10.1016/j.clnu.2023.12.017

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

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

Authors

Michael Wilschanski¹, Anne Munck², Estefania Carrion³, Marco Cipolli⁴, Sarah Collins⁵, Carla Colombo⁶, Dimitri Declercq⁷, Elpis Hatziagorou⁸, Jessie Hulst⁹, Daina Kalnins¹⁰, Christina N Katsagoni¹¹, Jochen G Mainz¹², Carmen Ribes-Koninckx¹³, Chris Smith¹⁴, Thomas Smith¹⁵, Stephanie Van Biervliet¹⁶, Michael Chourdakis¹⁷

Affiliations

¹ Pediatric Gastroenterology, Hadassah Hebrew University Medical Center, Jerusalem, Israel. Electronic address: michaelwil@hadassah.org.il.
² Cystic Fibrosis Centre, Hopital Necker-Enfants Malades, AP-HP, Paris, France.
³ Division of Gastroenterology, Hepatology and Nutrition, The Hospital for Sick Children, Toronto, Canada.
⁴ Cystic Fibrosis Center, Azienda Ospedaliera Universitaria Integrata, Verona, Italy.
⁵ CF Therapies Team, Royal Brompton & Harefield Hospital, London, UK.
⁶ University of Milan, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milan, Italy.
⁷ Cystic Fibrosis Reference Centre, Ghent University Hospital and Department of Internal Medicine and Paediatrics, Faculty of Medicine and Health Sciences, Ghent University, Ghent, Belgium.
⁸ Cystic Fibrosis Unit, 3rd Pediatric Dept, Hippokration Hospital, Aristotle University of Thessaloniki, Greece.
⁹ Division of Gastroenterology, Hepatology and Nutrition, The Hospital for Sick Children, Toronto, Canada; Department of Pediatrics and Department of Nutritional Sciences, The University of Toronto, Toronto, Canada.
¹⁰ Department of Clinical Dietetics, The Hospital for Sick Children, Toronto, Canada.
¹¹ Department of Clinical Nutrition, Agia Sofia Children's Hospital, Athens, Greece; EFAD, European Specialist Dietetic Networks (ESDN) for Gastroenterology, Denmark.
¹² Brandenburg Medical School, University Hospital. Klinikum Westbrandenburg, Brandenburg an der Havel, Germany.
¹³ Pediatric Gastroenterology and Paediatric Cystic Fibrosis Unit. La Fe Hospital & La Fe Research Institute, Valencia, Spain.
¹⁴ Department of Dietetics, Royal Alexandra Children's Hospital, Brighton, UK.
¹⁵ Independent Patient Consultant Working at Above-disease Level, UK.
¹⁶ Paediatric Gastroenterology, Hepatology and Nutrition, Ghent University Hospital, Belgium.
¹⁷ School of Medicine, Faculty of Health Sciences, Aristotle University of Thessaloniki, Greece.

PMID: 38169175
DOI: 10.1016/j.clnu.2023.12.017

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.

MeSH terms

Adult
Child
Cystic Fibrosis* / therapy
Humans
Infant
Nutrition Therapy*
Nutritional Status
Vitamin A
Vitamins

Substances

Vitamins
Vitamin A