Society guideline links: Cystic fibrosis

Introduction — 

This topic includes links to society and government-sponsored guidelines from selected countries and regions around the world. We will update these links periodically; newer versions of some guidelines may be available on each society's website. Some societies may require users to log in to access their guidelines.

The recommendations in the following guidelines may vary from those that appear in UpToDate topic reviews. Readers who are looking for UpToDate topic reviews should use the UpToDate search box to find the relevant content.

Links to related guidelines are provided separately:

●(See "Society guideline links: Hemoptysis".)

●(See "Society guideline links: Nontuberculous mycobacteria".)

●(See "Society guideline links: Pediatric liver disease".)

●(See "Society guideline links: Chronic pancreatitis and pancreatic exocrine insufficiency".)

●(See "Society guideline links: Lung transplantation".)

●(See "Society guideline links: Bronchiectasis".)

●(See "Society guideline links: Portal hypertension and ascites".)

International

●International Society for Pediatric and Adolescent Diabetes (ISPAD): Clinical practice consensus guidelines for the management of cystic fibrosis-related diabetes in children and adolescents (2022)

●International Committee on Mental Health in Cystic Fibrosis (ICMH-CF): Cystic Fibrosis Foundation and European Cystic Fibrosis Society consensus statements for screening and treating depression and anxiety (2016, reviewed 2021)

Canada

●European Society for Paediatric Gastroenterology, Hepatology, and Nutrition (ESPGHAN) and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition (NASPGHAN): Position paper towards a standardized classification of the hepatobiliary manifestations in cystic fibrosis (CFHBI) (2024)

●Choosing Wisely Canada: Don't delay conversations about wishes and goals with patients who have serious or progressive chronic respiratory illness, such as COPD, IPF, PH, or CF, or advanced neuromuscular disease affecting the respiratory system (e.g. Duchenne Muscular Dystrophy, ALS, etc.). Having and documenting these conversations can help to avoid unwanted and/or unnecessary and potentially harmful interventions. (2023)

●ESPGHAN and NASPGHAN: Position paper on the nutritional considerations in pediatric pancreatitis (2018)

●ESPGHAN and NASPGHAN: Clinical report on the assessment of exocrine pancreatic function and pancreatitis in children (2015)

United States

●Cystic Fibrosis Foundation (CFF): Clinical care guidelines

•Age-specific care guidelines

-Evidence-based guideline for the management of CRMS/CFSPID (2024)

•CF-related conditions

-Consensus recommendations on cystic fibrosis screening, evaluation, and management of hepatobiliary disease (2024)

-Clinical care guidelines for cystic fibrosis-related diabetes (2010, reviewed 2021)

-Consensus statements for screening and treating depression and anxiety (2016, reviewed 2021)

•Diagnosis care guidelines

-Consensus guidelines on the diagnosis of cystic fibrosis (2017, reviewed 2021)

•Infection prevention and control care guidelines

-Consensus recommendations for the management of non-tuberculous mycobacteria in individuals with cystic fibrosis (2016, reviewed 2021)

•Nutrition and GI care guidelines

•Respiratory care guidelines

-Pulmonary guidelines on use of cystic fibrosis transmembrane conductance regulator modulator therapy in patients with cystic fibrosis (2018, reviewed 2021)

●European Society for Paediatric Gastroenterology, Hepatology, and Nutrition (ESPGHAN) and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition (NASPGHAN): Position paper towards a standardized classification of the hepatobiliary manifestations in cystic fibrosis (CFHBI) (2024)

●American College of Medical Genetics and Genomics (ACMG): A position paper on the updated recommendations for CFTR carrier screening (2023)

●American College of Obstetricians and Gynecologists (ACOG): Committee opinion on carrier screening for genetic conditions (2017, reaffirmed 2023)

●American Gastroenterological Association (AGA): Clinical practice update on the epidemiology, evaluation, and management of exocrine pancreatic insufficiency – Expert review (2023)

●Academy of Nutrition and Dietetics (AND): Cystic fibrosis (CF) guideline (2020)

●ACMG: Technical standard on CFTR variant testing (2020)

●European Society for Paediatric Gastroenterology, Hepatology, and Nutrition (ESPGHAN) and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition (NASPGHAN): Position paper on the nutritional considerations in pediatric pancreatitis (2018)

●ESPGHAN and NASPGHAN: Clinical report on the assessment of exocrine pancreatic function and pancreatitis in children (2015)

●American Thoracic Society (ATS): An official workshop report on optimal lung function tests for monitoring cystic fibrosis, bronchopulmonary dysplasia, and recurrent wheezing in children less than 6 years of age (2013)

Europe

●European Society for Paediatric Gastroenterology, Hepatology, and Nutrition (ESPGHAN) and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition (NASPGHAN): Position paper towards a standardized classification of the hepatobiliary manifestations in cystic fibrosis (CFHBI) (2024)

●Cystic Fibrosis Foundation (CFF) and European Cystic Fibrosis Society (ECFS): Consensus recommendations for the management of non-tuberculous mycobacteria in individuals with cystic fibrosis (2016, reviewed 2021)

●ESPGHAN and NASPGHAN: Position paper on the nutritional considerations in pediatric pancreatitis (2018)

●ECFS: Best practice guidelines (2018)

●European Society for Clinical Nutrition and Metabolism (ESPEN)-ESPGHAN-ECFS: Guidelines on nutrition care for infants, children, and adults with cystic fibrosis (2016)

●ESPGHAN and NASPGHAN: Clinical report on the assessment of exocrine pancreatic function and pancreatitis in children (2015)

●European Respiratory Society (ERS)/ECFS: Report on the care of adults with cystic fibrosis (2015)

●ECFS: Best practice guidance for the diagnosis and management of cystic fibrosis-associated liver disease (2011)

●ECFS: European cystic fibrosis bone mineralisation guidelines (2011)

●ECFS: Guideline on end of life care for patients with cystic fibrosis (2011)

●ECFS: Guidelines for the diagnosis and management of distal intestinal obstruction syndrome in cystic fibrosis patients (2011)

●ECFS: Guiding principles on how to manage relevant psychological aspects within a CF team – Interdisciplinary approaches (2011)

●ESPGHAN: Defining DIOS and constipation in cystic fibrosis with a multicentre study on the incidence, characteristics, and treatment of DIOS (2010)

●ECFS: European best practice guidelines for cystic fibrosis neonatal screening (2009)

●ECFS: Guidelines for the management of pregnancy in women with cystic fibrosis (2008)

United Kingdom

●Cystic Fibrosis Trust: Consensus documents

●National Institute for Health and Care Excellence (NICE): Quality standard on cystic fibrosis (2018)

●NICE: Guideline on cystic fibrosis – Diagnosis and management (2017)

●Royal College of Paediatrics and Child Health (RCPCH): Guidelines for the performance of the sweat test for the investigation of cystic fibrosis in the UK, 2nd version (2014)

Australia–New Zealand

●Cystic Fibrosis Australia (CFA): Standards of care for cystic fibrosis (2023)

●Thoracic Society of Australia and New Zealand (TSANZ): Position paper on work environment risks for health care workers with cystic fibrosis (2018)

●TSANZ: Nutrition guidelines for cystic fibrosis in Australia and New Zealand (2017)

●TSANZ: Clinical practice guideline on physiotherapy for cystic fibrosis in Australia and New Zealand (2016)

●Asian Pacific Society of Respirology (APSR): Clinical practice guideline on Australian standards of care for cystic fibrosis-related diabetes (2013)