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 (2015)
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)
●Canadian Paediatric Society (CPS): A call for action – Recommendations to improve transition to adult care for youth with complex health care needs (2022)
●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
•CFTR modulator therapy care guidelines
•Diagnosis clinical care guidelines
•Infection prevention and control care guidelines
•Nutrition and GI care guidelines
•Other CF-related conditions care guidelines
•Palliative care guidelines
•Respiratory care guidelines
•Screening and treating depression and anxiety guidelines
●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 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)
●American Thoracic Society (ATS): Clinical practice guideline on home oxygen therapy for children (2018)
●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)
●American College of Obstetricians and Gynecologists (ACOG): Committee opinion on carrier screening for genetic conditions (2017)
●CFF and European Cystic Fibrosis Society (ECFS): Consensus recommendations for the management of non-tuberculous mycobacteria in individuals with cystic fibrosis (2015)
●ESPGHAN and NASPGHAN: Clinical report on the assessment of exocrine pancreatic function and pancreatitis in children (2015)
●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)
●American College of Chest Physicians (CHEST): Evidence-based clinical practice guidelines for monitoring of nonsteroidal immunosuppressive drugs in patients with lung disease and lung transplant recipients (2012)
●American Diabetes Association (ADA) and CFF: Clinical care guidelines for cystic fibrosis-related diabetes (2010)
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)
●European position paper on rhinosinusitis and nasal polyps (2020)
●European Respiratory Society (ERS)/Thoracic Society of Australia and New Zealand (TSANZ): Statement on the management of reproduction and pregnancy in women with airways diseases (2020)
●ESPGHAN and NASPGHAN: Position paper on the nutritional considerations in pediatric pancreatitis (2018)
●European Cystic Fibrosis Society (ECFS): Best practice guidelines (2018)
●Cystic Fibrosis Foundation (CFF) and ECFS: Consensus recommendations for the management of non-tuberculous mycobacteria in individuals with cystic fibrosis (2016)
●ERS/ECFS: Report on the care of adults with cystic fibrosis (2016)
●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)
●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)
●European Respiratory Society (ERS)/Thoracic Society of Australia and New Zealand (TSANZ): Statement on the management of reproduction and pregnancy in women with airways diseases (2020)
●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)
