Le diverse figure professionali dell’UOS-FC sono state formate nella lettura delle linee guida nazionali e internazionali. La gestione del paziente segue tali linee guida. In tal caso l’orientamento è quello di applicare linee guida che abbiano la migliore qualità delle prove e la maggiore forza delle raccomandazioni. A questo si aggiunge il costante aggiornamento attraverso gli studi di metanalisi (Cochrane) e revisione sistematica della letteratura (www.cfdb.eu)
PRINCIPALI LINEE GUIDA
Smyth AR et al. European Cystic Fibrosis Society. European Cystic Fibrosis Society Standards of Care: Best Practice guidelines. J Cyst Fibros. 2014;13:S23-42
Stern M et al. European Cystic Fibrosis Society Standards of Care: Quality Management in cystic fibrosis. J Cyst Fibros. 2014;13:S43-59.
Conway S et al. European Cystic Fibrosis Society Standards of Care: Framework for the Cystic Fibrosis Centre. J Cyst Fibros. 2014;13:S3-22.
McColley SA et al. Practice guidelines, clinical trials, and unexpected results in cystic fibrosis. Ann Am Thorac Soc. 2014;11:402-3.
Lahiri T et al. Clinical Practice Guidelines From the Cystic Fibrosis Foundation for Preschoolers With Cystic Fibrosis. Pediatrics. 2016;137. pii: e20151784.
Castellani C. et al. ECFS best practice guide lines: 2018 revision. Journal of Cystic Fibrosis 2018; 17:153 178
Genetica e Diagnosi
Farrell PM et al. Diagnosis of Cystic Fibrosis: Consensus Guidelines from the Cystic Fibrosis Foundation. J Pediatr. 2017;181:S4-S15.
Langfelder-Schwind E et al. Molecular testing for cystic fibrosis carrier status practice guidelines: recommendations of the National Society of Genetic Counselors. J Genet Couns. 2014;23:5-15.
Bombieri C et al. Recommendations for the classification of diseases as CFTR-related disorders.J Cyst Fibros. 2011;10:S86-102.
Dequeker E et al. Best practice guidelines for molecular genetic diagnosis of Cystic Fibrosis and CFTR-related disorders-updated European reccomendations. Eur J Hum Genet. 2009;17:51-65.
Gruppo di lavoro SIFC sul Test del Sudore. Test del sudore: raccomandazioni per una corretta esecuzione ed interpretazione dei risultati. Settembre 2017. www.sifc.it
Gruppo Professionale Genetisti SIFC. Modelli di analisi genetica per la Fibrosi Cistica. 2005.www.sifc.it
Nutrizione e Gastroenterologia
Turck D et al. ESPEN-ESPGHAN-ECFS guidelines on nutrition care for infants, children, and adults with cystic fibrosis. Clin Nutr. 2016;35:557-77.
Tangpricha V et al. Cystic Fibrosis Foundation Vitamin D Evidence-Based Review Committee. An update on the screening, diagnosis, management, and treatment of vitamin D deficiency in individuals with cystic fibrosis: evidence-based recommendations from the Cystic Fibrosis Foundation. J Clin Endocrinol Metab. 2012;97:1082-93.
Colombo C et al. ECFS. Guidelines for the diagnosis and management of distal intestinal obstruction syndrome in cystic fibrosis patients. J Cyst Fibros. 2011;10:S24-8.
Debray D et al. Best practice guidance for the diagnosis and management of cystic fibrosis-associated liver disease. J Cyst Fibros. 2011;10:S29-36.
Diabete
Moran A et al. International Society for Pediatric and Adolescent Diabetes. ISPAD Clinical Practice Consensus Guidelines 2014. Management of cystic fibrosis-related diabetes in children and adolescents. Pediatr Diabetes. 2014;15 S20:65-76.
Middleton PG et al. Australian standards of care for cystic fibrosis-related diabetes. Respirology. 2014;19:185-92.
Moran A et al. CFRD Guidelines Committee. Clinical care guidelines for cystic fibrosis-related diabetes: a position statement of the American Diabetes Association and a clinical practice guideline of the Cystic Fibrosis Foundation, endorsed by the Pediatric Endocrine Society. Diabetes Care. 2010;33:2697-708.
Complicanze polmonari
Flume PA et al. Clinical Practice Guidelines for Pulmonary Therapies Committee; Cystic Fibrosis Foundation Pulmonary Therapies Committee. Cystic fibrosis pulmonary guidelines: pulmonary complications: hemoptysis and pneumothorax. Am J Respir Crit Care Med. 2010;182:298-306.
Lord RW et al. Pneumothorax in cystic fibrosis: beyond the guidelines. Paediatr Respir Rev. 2016;20:30-3.
Stevens DA et al. Allergic bronchopulmonary aspergillosis in cystic fibrosis-state of the art: Cystic Fibrosis Foundation Consensus Conference. Clin Infect Dis. 2003;37:S225-64.
Infezioni
Saiman L et al. Cystic Fibrous Foundation; Society for Healthcare Epidemiology of America. Infection prevention and control guideline for cystic fibrosis: 2013 update. Infect Control Hosp Epidemiol. 2014;35:S1-S67.
Schaffer K et al. Epidemiology of infection and current guidelines for infection prevention in cystic fibrosis patients. J Hosp Infect. 2015;89:309-13.
Langan KM et al. Managing Pseudomonas aeruginosa respiratory infections in cystic fibrosis. Curr Opin Infect Dis. 2015;28:547-56.
Mogayzel PJ Jr et al. Cystic Fibrosis Foundation Pulmonary Clinical Practice Guidelines Committee. Cystic Fibrosis Foundation pulmonary guideline. Pharmacologic approaches to prevention and eradication of initial Pseudomonas aeruginosa infection. Ann Am Thorac Soc. 2014;11:1640-50
Abely M et al. National consensus regarding azithromycin use in cystic fibrosis. Rev Mal Respir. 2015;32:557-65.
Floto RA et al. US Cystic Fibrosis Foundation and European Cystic Fibrosis Society consensus recommendations for the management of non-tuberculous mycobacteria in individuals with cystic fibrosis. Thorax. 2016;71:1-22.
Floto RA et al. US Cystic Fibrosis Foundation and European Cystic Fibrosis Society consensus recommendations for the management of non-tuberculous mycobacteria in individuals with cystic fibrosis: executive summary. Thorax. 2016;71:88-90.
Flume PA et al. US Cystic Fibrosis Foundation and European Cystic Fibrosis Society consensus recommendations for the management of non-tuberculous mycobacteria in individuals with cystic fibrosis. J Cyst Fibros. 2016;15:139-40.
Gruppo di Lavoro della Società Italiana di Fibrosi Cistica. Raccomandazioni per la prevenzione ed il controllo delle infezioni da patogeni respiratori in fibrosi cistica. 2006. www.sifc.it.
Microbiologia
Coman G et al. Current microbiological data on lower respiratory tract infection in cystic fibrosis. Part II: Recommendations for microbiological diagnosis in cystic fibrosis. Rev Med Chir Soc Med Nat Iasi. 2012;116:898-901.
Laboratory Standards for Processing Microbiological Samples from People with Cystic Fibrosis. First Edition.2010.http://cftrust.org.uk/
Gruppo professionale Microbiologi SIFC. Linee Guida per la corretta esecuzione delle indagini microbiologiche relative a pazienti con fibrosi cistica. 2006 www.sifc.it
Screening e CFSPID
Sermet-Gaudelus I et al. Management of infants whose diagnosis is inconclusive at neonatal screening for cystic fibrosis. Arch Pediatr. 2017;24:401-414.
Munck A et al. Cystic Fibrosis Screen Positive, Inconclusive Diagnosis (CFSPID): A new designation and management recommendations for infants with an inconclusive diagnosis following newborn screening. J Cyst Fibros. 2015;14:706-13.
Sermet-Gaudelus I et al. Guidelines on the early management of infants diagnosed with cystic fibrosis following newborn screening. J Cyst Fibros. 2010;9:323-9.
Castellani C et al. European best practice guidelines for cystic fibrosis neonatal screening. J Cyst Fibros. 2009;8:153-73.
Fisioterapia
Button BM et al. Physiotherapy for cystic fibrosis in Australia and New Zealand: A clinical practice guideline. Respirology. 2016;21:656-67.
Hebestreit H et al. European Cystic Fibrosis Exercise Working Group. Statement on Exercise Testing in Cystic Fibrosis. Respiration. 2015;90:332-51.
Flume PA et al. Clinical Practice Guidelines for Pulmonary Therapies Committee. Airway Clearance Therapies Respir Care. 2009;54:522-537.
Psicologia
Quittner AL et al. International Committee on Mental Health in Cystic Fibrosis: Cystic Fibrosis Foundation and European Cystic Fibrosis Society consensus statements for screening and treating depression and anxiety. Thorax. 2016;71:26-34.
Trapiantologia
Hirche TO et al. Practical guidelines: lung transplantation in patients with cystic fibrosis. Pulm Med. 2014;2014:621342.
Weill D et al. A consensus document for the selection of lung transplant candidates: 2014–an update from the Pulmonary Transplantation Council of the International Society for Heart and Lung Transplantation. J Heart Lung Transplant. 2015;34:1-15.