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Pulmonology1796 papers

Cystic fibrosis

Last edited: 4/23/2026

Overview

Cystic fibrosis (CF) is a genetic disorder characterized by defective cystic fibrosis transmembrane conductance regulator (CFTR) protein, leading to thick mucus production, primarily affecting the lungs and pancreas, but also impacting multiple organ systems.

Diagnosis

Sweat chloride test: Elevated levels (≥60 mmol/L) confirm diagnosis 22.

Genetic testing: Identifies CFTR mutations 33.

Clinical features: Chronic respiratory infections, pancreatic insufficiency, and malnutrition 8.

Management

First-line treatments:

- CFTR modulators (e.g., elexacaftor/tezacaftor/ivacaftor): Improves lung function and nutritional status 1 23 25. - Antibiotics: For recurrent respiratory infections 3 7. - Pancreatic enzyme supplements: To aid digestion and nutrient absorption 8. - Fat-soluble vitamin supplementation: Particularly vitamins A, D, E, and K, often using specialized formulations 1 13 24 44.

Adjunctive treatments:

- Azithromycin: For chronic suppression of Pseudomonas aeruginosa 6 46. - Pulmonary rehabilitation: To improve exercise capacity and quality of life 38. - Pain management: Evidence-informed guidelines address comprehensive pain management strategies 5.

Special Populations

Pregnancy:

- Monitor for adverse drug reactions; pharmacovigilance is crucial 2.

Pediatrics:

- Close monitoring of fat-soluble vitamin status and tailored supplementation protocols 1 25. - Nutritional support and growth monitoring essential 1 11 31 40.

Elderly:

- Focus on cardiometabolic risk factors and bone health with CFTR modulators 7.

Comorbidities:

- Venous thromboembolism (VTE) risk management is increasingly important 16. - Chronic rhinosinusitis management requires multidisciplinary care 30 57.

Key Recommendations

Implement CFTR modulators for eligible patients to improve lung function and nutritional status (Evidence: Strong 1 23).

Regular monitoring and supplementation of fat-soluble vitamins, especially in pediatric and adult patients, using optimized formulations (Evidence: Strong 1 13 24).

Use of azithromycin for chronic suppression of respiratory pathogens in appropriate patients (Evidence: Moderate 6 46).

Multidisciplinary care including dietitians and gastroenterologists to address nutritional deficiencies and digestive outcomes (Evidence: Expert opinion 4).

Comprehensive pain management strategies based on evidence-informed guidelines (Evidence: Moderate 5).

Enhanced vigilance for adverse drug reactions during pregnancy and psychiatric monitoring with CFTR modulator initiation (Evidence: Moderate 2 6).

References

Showing 100 most recent of 295 indexed papers.

1 Kimber F, Sills D. The Impact of Elexacaftor/Tezacaftor/Ivacaftor on Fat-Soluble Vitamin Status and Supplementation in a Pediatric Cystic Fibrosis Cohort. Pediatric pulmonology 2026. link 2 Abolhassani N, Dao K, Noseda R, Bedussi F, Ceschi A, Panchaud A et al.. CFTR during pregnancy and adverse drug reactions: A pharmacovigilance disproportionality analysis in VigiBase. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society 2026. link 3 Whiteside SA, McGinniss JE, Deek RA, Merenstein C, Britton N, Simon-Soro A et al.. Lung transplant for CF: Low lung bacterial burden and immune mediators in year one associate with CLAD development. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society 2026. link 4 Sankararaman S, Schindler T, Leonard A, Vavrina K, Bailey J, Thavamani A et al.. Collaboration between registered dietitians and gastroenterologists in cystic fibrosis care: Results of an international cross-sectional survey. Nutrition in clinical practice : official publication of the American Society for Parenteral and Enteral Nutrition 2025. link 5 Dellon EP, Allada G, Allgood SJ, Georgiopoulos AM, Goggin JL, Hadjiliadis D et al.. Addressing pain in people living with cystic fibrosis: Cystic fibrosis foundation evidence-informed guidelines. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society 2025. link 6 O'Connor J, Nazareth D, Wat D, Southern KW, Frost F. Regulatory adverse drug reaction analyses support a temporal increase in psychiatric reactions after initiation of cystic fibrosis combination modulator therapies. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society 2025. link 7 Gramegna A, Ruscica M, Leonardi G, Macchi C, Fichtner I, Putti G et al.. Cardiometabolic risk factors in adults with cystic fibrosis undergoing elexacaftor/tezacaftor/ivacaftor therapy. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society 2025. link 8 Wilschanski M, Munck A, Carrion E, Cipolli M, Collins S, Colombo C et al.. ESPEN-ESPGHAN-ECFS guideline on nutrition care for cystic fibrosis. Clinical nutrition (Edinburgh, Scotland) 2024. link 9 Green DM, Lahiri T, Raraigh KS, Ruiz F, Spano J, Antos N et al.. Cystic Fibrosis Foundation Evidence-Based Guideline for the Management of CRMS/CFSPID. Pediatrics 2024. link 10 Curatola A, Corona F, Squillaci D, Saccari A, Chiaretti A, Barbi E et al.. Lung ultrasound evaluation in people with cystic fibrosis: A new approach in the pulmonology outpatient clinic. Pediatric pulmonology 2024. link 11 Bodewes FAJA, Freeman AJ, Weymann A, Debray D, Scheers I, Verkade HJ et al.. Towards a Standardized Classification of the Hepatobiliary Manifestations in Cystic Fibrosis (CFHBI): A Joint ESPGHAN/NASPGHAN Position Paper. Journal of pediatric gastroenterology and nutrition 2024. link 12 Escobedo-Monge MF, Marcos-Temprano M, Parodi-Román J, Escobedo-Monge MA, Alonso-Vicente C, Torres-Hinojal MC et al.. Calcium, Phosphorus, and Vitamin D Levels in a Series of Cystic Fibrosis Patients: A Cross-Sectional Study. International journal of molecular sciences 2024. link 13 Soleimanzadeh M, Talebi S, Jaafari MR, Sayedi SJ, Emadzadeh M, Kianifar HR. Evaluation of the efficiency of nanomicellar formulation of fat-soluble vitamins in patients with cystic fibrosis: the study protocol for a randomized controlled trial. Trials 2024. link 14 Queiroz DJM, Silva AS, da Silva Júnior CC, Paiva MP, Assis CS, Persuhn DC et al.. Influence of the BsmI polymorphism of the vitamin D receptor gene on the levels of vitamin D, inflammatory and oxidative stress profile in patients with cystic fibrosis supplemented with Colecalciferol megadose. Clinical nutrition ESPEN 2024. link 15 Braun AT, Lai HJ, Laxova A, Biller JA, Hubertz EK, Zhao Z et al.. Vitamin D status and variable responses to supplements depend in part on genetic factors in adults with cystic fibrosis. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society 2024. link 16 Abbinanti A, Witt DM, Saunders J, Jones AE, Young DC. Venous thromboembolism management in people with cystic fibrosis. Pediatric pulmonology 2024. link 17 von Dossow V, Hulde N, Starke H, Schramm R. How Would We Treat Our Own Cystic Fibrosis With Lung Transplantation?. Journal of cardiothoracic and vascular anesthesia 2024. link 18 Dehbozorgi A, Jandali B, Turner R, Rohr A, Custer B, Young K et al.. Safety of non-cuffed tunneled central venous catheters in adults with cystic fibrosis. Respiratory medicine and research 2024. link 19 Okroglic L, Sohier P, Martin C, Lheure C, Franck N, Honoré I et al.. Acneiform Eruption Following Elexacaftor-Tezacaftor-Ivacaftor Treatment in Patients With Cystic Fibrosis. JAMA dermatology 2023. link 20 Dubin E, Lowers J, Dellon EP, Hempstead S, Faro A, Tallarico E et al.. Prevalence of unmet pain and symptom management needs in adults with cystic fibrosis. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society 2023. link 21 Bergeron C, Potter KJ, Boudreau V, Ouliass B, Bonhoure A, Lacombe J et al.. Low vitamin K status in adults with cystic fibrosis is associated with reduced body mass index, insulin secretion, and increased pseudomonal colonization. Applied physiology, nutrition, and metabolism = Physiologie appliquee, nutrition et metabolisme 2023. link 22 Iniesta RR, Cook S, Oversby G, Koufaki P, Van der Linden ML, Vlachopoulos D et al.. Systematic review and meta-analysis: Associations of vitamin D with pulmonary function in children and young people with cystic fibrosis. Clinical nutrition ESPEN 2023. link 23 Hergenroeder GE, Faino A, Bridges G, Bartlett LE, Cogen JD, Green N et al.. The impact of elexacaftor/tezacaftor/ivacaftor on fat-soluble vitamin levels in people with cystic fibrosis. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society 2023. link 24 Mangas-Sánchez C, Garriga-García M, Serrano-Nieto MJ, Garcia-Romero R, Álvarez Beltrán M, Crehuá-Gaudiza E et al.. Safety and efficacy of a new supplementation protocol in patients with cystic fibrosis and vitamin D deficiency. Anales de pediatria 2023. link 25 Schembri L, Warraich S, Bentley S, Carr SB, Balfour-Lynn IM. Impact of elexacaftor/tezacaftor/ivacaftor on fat-soluble vitamin levels in children with cystic fibrosis. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society 2023. link 26 Temur HO, Alkan A, Yozgat CY, Cakir E, Yazan H, Yabul FC et al.. MRI evaluation of right heart functions in children with mild cystic fibrosis. Cardiology in the young 2023. link 27 Hudson BN, Jacobs HR, Philbrick A, Zhou XA, Simonsen MM, Safirstein JA et al.. Drug-induced acne with elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society 2022. link 28 Ley D, Turck D. Digestive outcomes in Cystic fibrosis. Best practice & research. Clinical gastroenterology 2022. link 29 Leong S, Sharma RK, Safi C, DiMango E, Keating C, Gudis DA et al.. Association of Quality of Life Measures and Otolaryngologic Care in Cystic Fibrosis Patients. The Annals of otology, rhinology, and laryngology 2022. link 30 Kimple AJ, Senior BA, Naureckas ET, Gudis DA, Meyer T, Hempstead SE et al.. Cystic Fibrosis Foundation otolaryngology care multidisciplinary consensus recommendations. International forum of allergy & rhinology 2022. link 31 Obregon LL, Jeong K, Hoydich ZP, Yabes J, Pilewski J, Richless C et al.. Associations between demographic characteristics and unmet supportive care needs in adults with cystic fibrosis. BMJ supportive & palliative care 2022. link 32 Vijayavel N, Koh SW, Goodman EL. Cystic fibrosis associated with Wernicke's encephalopathy in an older adult. BMJ case reports 2022. link 33 Wysocka-Wojakiewicz P, Woś H, Wielkoszyński T, Pyziak-Skupień A, Grzybowska-Chlebowczyk U. Vitamin Status in Children with Cystic Fibrosis Transmembrane Conductance Regulator Gene Mutation. Nutrients 2022. link 34 Lai HJ, Song J, Lu Q, Murali SG, Gajapathy M, Wilk BM et al.. Genetic factors help explain the variable responses of young children with cystic fibrosis to vitamin D supplements. Clinical nutrition ESPEN 2022. link 35 Sankararaman S, Hendrix SJ, Schindler T. Update on the management of vitamins and minerals in cystic fibrosis. Nutrition in clinical practice : official publication of the American Society for Parenteral and Enteral Nutrition 2022. link 36 Farahbakhsh N, Bagherian N, Shabanpourhaghighi S, Khalilzadeh S, Tabatabaii SA, Khanbabaee G. Corneal opacification, an atypical presentation of cystic fibrosis: a case report and review of the literature. Journal of medical case reports 2022. link 37 Sathe M, Sharma PB, Savant AP. Year in review 2020: Nutrition and gastrointestinal disease in cystic fibrosis. Pediatric pulmonology 2021. link 38 Sharma PB, Sathe M, Savant AP. Year in Review 2020: Multisystemic impact of cystic fibrosis. Pediatric pulmonology 2021. link 39 Lusman SS, Borowitz D, Marshall BC, Narkewicz MR, Gonska T, Grand RJ et al.. DIGEST: Developing innovative gastroenterology specialty training. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society 2021. link 40 Shaikhkhalil AK, Freeman AJ, Sathe M. Variations in Nutrition Practices in Cystic Fibrosis: A Survey of the DIGEST Program. Nutrition in clinical practice : official publication of the American Society for Parenteral and Enteral Nutrition 2021. link 41 Oates G, Rutland S, Juarez L, Friedman A, Schechter MS. The association of area deprivation and state child health with respiratory outcomes of pediatric patients with cystic fibrosis in the United States. Pediatric pulmonology 2021. link 42 Bhimavarapu A, Deng Q, Bean M, Lee N, Ziegler TR, Alvarez J et al.. Factors Contributing to Vitamin D Status at Hospital Admission for Pulmonary Exacerbation in Adults With Cystic Fibrosis. The American journal of the medical sciences 2021. link 43 Victoria CB, Casilda O, Nuria P, José AF, María GO, José SF et al.. Oral Nutritional Supplements in Adults with Cystic Fibrosis: Effects on Intake, Levels of Fat-Soluble Vitamins, and Bone Remodeling Biomarkers. Nutrients 2021. link 44 Nowak JK, Sobkowiak P, Drzymała-Czyż S, Krzyżanowska-Jankowska P, Sapiejka E, Skorupa W et al.. Fat-Soluble Vitamin Supplementation Using Liposomes, Cyclodextrins, or Medium-Chain Triglycerides in Cystic Fibrosis: A Randomized Controlled Trial. Nutrients 2021. link 45 Song Y, Palacios AC, Thiagalingam A, Middleton PG. Azithromycin and tezacaftor/ivacaftor is associated with first-degree heart block in an adult with cystic fibrosis. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society 2021. link 46 Magaret AS, Salerno J, Deen JF, Kloster M, Mayer-Hamblett N, Ramsey BW et al.. Long-term azithromycin use is not associated with QT prolongation in children with cystic fibrosis. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society 2021. link 47 Jayawardena ADL, Fracchia MS, Bartley BL, Yonker LM, Lapey A, Virgin F et al.. Working towards consensus in the management of pediatric chronic rhinosinusitis in cystic fibrosis. International journal of pediatric otorhinolaryngology 2020. link 48 Farrell MH, Sims AM, La Pean Kirschner A, Farrell PM, Tarini BA. Vulnerable Child Syndrome and Newborn Screening Carrier Results for Cystic Fibrosis or Sickle Cell. The Journal of pediatrics 2020. link 49 Omer S, Koumangoye R, Delpire E. A mutation in the Na-K-2Cl cotransporter-1 leads to changes in cellular metabolism. Journal of cellular physiology 2020. link 50 Tham A, Katz TE, Sutherland RE, Garg M, Liu V, Tong CW et al.. Micronutrient intake in children with cystic fibrosis in Sydney, Australia. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society 2020. link 51 El-Koofy N, El-Mahdy M, Fathy M, El Falaki M, El Dine Hamed DH. Nutritional rehabilitation for children with cystic fibrosis: Single center study. Clinical nutrition ESPEN 2020. link 52 Okebukola PO, Kansra S, Barrett J. Vitamin E supplementation in people with cystic fibrosis. The Cochrane database of systematic reviews 2020. link 53 Loukou I, Moustaki M, Sardeli O, Plyta M, Douros K. Association of vitamin D status with lung function measurements in children and adolescents with cystic fibrosis. Pediatric pulmonology 2020. link 54 Jagannath VA, Thaker V, Chang AB, Price AI. Vitamin K supplementation for cystic fibrosis. The Cochrane database of systematic reviews 2020. link 55 Liu CZ, Li FY, Lv XF, Ma MM, Li XY, Lin CX et al.. Endophilin A2 regulates calcium-activated chloride channel activity via selective autophagy-mediated TMEM16A degradation. Acta pharmacologica Sinica 2020. link 56 Scaravilli V, Morlacchi LC, Merrino A, Piacentino E, Marasco D, Zanella A et al.. Intraoperative extracorporeal membrane oxygenation for lung transplantation in cystic fibrosis patients: Predictors and impact on outcome. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society 2020. link 57 Lowery AS, Gallant JN, Woodworth BA, Brown RF, Sawicki GS, Shannon CN et al.. Chronic rhino-sinusitis treatment in children with cystic fibrosis: A cross-sectional survey of pediatric pulmonologists and otolaryngologists. International journal of pediatric otorhinolaryngology 2019. link 58 You-Ten KE, Zasso FB. Transnasal Humidified Rapid-Insufflation Ventilatory Exchange for Deep Sedation in a Lung Transplant Candidate With Severe Cystic Fibrosis: A Case Report. A&A practice 2019. link 59 Léveillé P, Knoth IS, Denis MH, Morin G, Barlaam F, Nyalendo C et al.. Association between fat-soluble nutrient status and auditory and visual related potentials in newly diagnosed non-screened infants with cystic fibrosis: A case-control study. Prostaglandins, leukotrienes, and essential fatty acids 2019. link 60 Abu-Fraiha Y, Elyashar-Earon H, Shoseyov D, Cohen-Cymberknoh M, Armoni S, Kerem E et al.. Increasing Vitamin D Serum Levels Is Associated With Reduced Pulmonary Exacerbations in Patients With Cystic Fibrosis. Journal of pediatric gastroenterology and nutrition 2019. link 61 Janzen KM, Sakon C, Lehman A, Sommer B, Brown C. High-dose Cholecalciferol Supplementation in Adults with Cystic Fibrosis. Pharmacotherapy 2019. link 62 Schapkaitz E, Weil R, White D, Klugman S. Asymptomatic catheter-related venous thrombosis in a child with cystic fibrosis: When to treat?. Journal of vascular nursing : official publication of the Society for Peripheral Vascular Nursing 2019. link 63 Li YG, Bisson A, Bodin A, Herbert J, Grammatico-Guillon L, Joung B et al.. C. Journal of the American Heart Association 2019. link 64 Zhu Y, Shudo Y, Lee AM, Woo YJ. Successful heart-lung-kidney and domino heart transplantation following veno-venous extracorporeal membrane oxygenation support. Interactive cardiovascular and thoracic surgery 2019. link 65 Savant AP, McColley SA. Cystic fibrosis year in review 2017. Pediatric pulmonology 2018. link 66 Hadjiliadis D, Khoruts A, Zauber AG, Hempstead SE, Maisonneuve P, Lowenfels AB. Cystic Fibrosis Colorectal Cancer Screening Consensus Recommendations. Gastroenterology 2018. link 67 Kirkwood ZI, Millar BC, Downey DG, Moore JE. Antimycobacterial activity of nonantibiotics associated with the polypharmacy of cystic fibrosis (CF) against mycobacterium abscessus. International journal of mycobacteriology 2018. link 68 de Vries JJ, Chang AB, Bonifant CM, Shevill E, Marchant JM. Vitamin A and beta (β)-carotene supplementation for cystic fibrosis. The Cochrane database of systematic reviews 2018. link 69 Sagel SD, Khan U, Jain R, Graff G, Daines CL, Dunitz JM et al.. Effects of an Antioxidant-enriched Multivitamin in Cystic Fibrosis. A Randomized, Controlled, Multicenter Clinical Trial. American journal of respiratory and critical care medicine 2018. link 70 Ziegler B, Perin C, Casarotto FC, Fagondes SC, Menna-Barreto SS, Dalcin PTR. Pulmonary hypertension as estimated by Doppler echocardiography in adolescent and adult patients with cystic fibrosis and their relationship with clinical, lung function and sleep findings. The clinical respiratory journal 2018. link 71 Mandal A, Priyadarshi M, Jat K, Kabra SK. Infantile Tremor Syndrome and Subdural Hemorrhage in an Infant with Cystic Fibrosis. Journal of tropical pediatrics 2017. link 72 Aralica M, Krleza JL. Evaluating performance in sweat testing in medical biochemistry laboratories in Croatia. Biochemia medica 2017. link 73 Chen E, Killeen KM, Peterson SJ, Saulitis AK, Balk RA. Evaluation of Pain, Dyspnea, and Goals of Care Among Adults With Cystic Fibrosis: A Comprehensive Palliative Care Survey. The American journal of hospice & palliative care 2017. link 74 Jagannath VA, Thaker V, Chang AB, Price AI. Vitamin K supplementation for cystic fibrosis. The Cochrane database of systematic reviews 2017. link 75 Okebukola PO, Kansra S, Barrett J. Vitamin E supplementation in people with cystic fibrosis. The Cochrane database of systematic reviews 2017. link 76 Pincikova T, Paquin-Proulx D, Sandberg JK, Flodström-Tullberg M, Hjelte L. Clinical impact of vitamin D treatment in cystic fibrosis: a pilot randomized, controlled trial. European journal of clinical nutrition 2017. link 77 Savant AP, McColley SA. Pediatric Pulmonology year in review 2015: Part 4. Pediatric pulmonology 2016. link 78 Zemanick ET, Ong T, Daines CL, Dellon EP, Muhlebach MS, Esther CR. Highlights from the 2015 North American Cystic Fibrosis Conference. Pediatric pulmonology 2016. link 79 Elborn JS, Bell SC, Madge SL, Burgel PR, Castellani C, Conway S et al.. Report of the European Respiratory Society/European Cystic Fibrosis Society task force on the care of adults with cystic fibrosis. The European respiratory journal 2016. link 80 Bari O, Paravar T. Isotretinoin therapy for the treatment of acne in patients with cystic fibrosis: a case series and review of the literature. Dermatology online journal 2016. link 81 Morin G, Orlando V, St-Martin Crites K, Patey N, Mailhot G. Vitamin D attenuates inflammation in CFTR knockdown intestinal epithelial cells but has no effect in cells with intact CFTR. American journal of physiology. Gastrointestinal and liver physiology 2016. link 82 Simoneau T, Sawicki GS, Milliren CE, Feldman HA, Gordon CM. A randomized controlled trial of vitamin D replacement strategies in pediatric CF patients. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society 2016. link 83 Ratjen F, Waters V, Klingel M, McDonald N, Dell S, Leahy TR et al.. Changes in airway inflammation during pulmonary exacerbations in patients with cystic fibrosis and primary ciliary dyskinesia. The European respiratory journal 2016. link 84 Coolen N, Gouya H, Kanaan R, Honoré I, Chapron J, Hubert D et al.. Renin-associated hypertension after bronchial artery embolization in cystic fibrosis. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society 2016. link 85 Lenehan PJ, Schramm CM, Collins MS. An evaluation strategy for potential QTc prolongation with chronic azithromycin therapy in cystic fibrosis. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society 2016. link 86 Savant AP, McColley SA. 2014 year in review: Cystic fibrosis. Pediatric pulmonology 2015. link 87 Burgel PR, Bellis G, Olesen HV, Viviani L, Zolin A, Blasi F et al.. Future trends in cystic fibrosis demography in 34 European countries. The European respiratory journal 2015. link 88 Lansing AH, McDonald C, Patel RA, Meihls S, Crowell K, Chatfield B et al.. Vitamin D deficiency in pediatric patients with cystic fibrosis: associated risk factors in the northern United States. Southern medical journal 2015. link 89 Cottam ST, Connett GJ. Routine use of daily oral vitamin K to treat infants with cystic fibrosis. Paediatric respiratory reviews 2015. link 90 Jagannath VA, Fedorowicz Z, Thaker V, Chang AB. Vitamin K supplementation for cystic fibrosis. The Cochrane database of systematic reviews 2015. link 91 Lee MJ, Kearns MD, Smith EM, Hao L, Ziegler TR, Alvarez JA et al.. Free 25-Hydroxyvitamin D Concentrations in Cystic Fibrosis. The American journal of the medical sciences 2015. link 92 Roberts R, Speight L, Lee J, George L, Ketchell RI, Lau D et al.. Retinal screening of patients with cystic fibrosis-related diabetes in Wales -- a real eye opener. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society 2015. link 93 Otani S, Westall GP, Levvey BJ, Marasco S, Lyon S, Snell GI. Managing central venous obstruction in cystic fibrosis recipients--lung transplant considerations. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society 2015. link 94 Visschers NC, Hulzebos EH, van Brussel M, Takken T. Comparing four non-invasive methods to determine the ventilatory anaerobic threshold during cardiopulmonary exercise testing in children with congenital heart or lung disease. Clinical physiology and functional imaging 2015. link 95 Cunningham F, Lewis S, Curnow L, Glazner J, Massie J. Respiratory physicians and clinic coordinators' attitudes to population-based cystic fibrosis carrier screening. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society 2014. link 96 Williams SN, Nussbaum E, Chin TW, Do PC, Singh KE, Randhawa I. Diagnosis of cystic fibrosis in the kindred of an infant with CFTR-related metabolic syndrome: importance of follow-up that includes monitoring sweat chloride concentrations over time. Pediatric pulmonology 2014. link 97 Nebbioso M, Quattrucci S, Leggieri E, Spadea L, Vingolo EM. Cystic fibrosis and new trends by ophthalmological evaluation: a pilot study. BioMed research international 2014. link 98 Lourenço BM, Costa KM, da Silva Filho M. Voice disorder in cystic fibrosis patients. PloS one 2014. link 99 Bonifant CM, Shevill E, Chang AB. Vitamin A supplementation for cystic fibrosis. The Cochrane database of systematic reviews 2014. link 100 Rana M, Wong-See D, Katz T, Gaskin K, Whitehead B, Jaffe A et al.. Fat-soluble vitamin deficiency in children and adolescents with cystic fibrosis. Journal of clinical pathology 2014. link

Original source

[1]
The Impact of Elexacaftor/Tezacaftor/Ivacaftor on Fat-Soluble Vitamin Status and Supplementation in a Pediatric Cystic Fibrosis Cohort. — Kimber F, Sills D Pediatric pulmonology (2026)
[2]
CFTR during pregnancy and adverse drug reactions: A pharmacovigilance disproportionality analysis in VigiBase. — Abolhassani N, Dao K, Noseda R, Bedussi F, Ceschi A, Panchaud A et al. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society (2026)
[3]
Lung transplant for CF: Low lung bacterial burden and immune mediators in year one associate with CLAD development. — Whiteside SA, McGinniss JE, Deek RA, Merenstein C, Britton N, Simon-Soro A et al. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society (2026)
[4]
Collaboration between registered dietitians and gastroenterologists in cystic fibrosis care: Results of an international cross-sectional survey. — Sankararaman S, Schindler T, Leonard A, Vavrina K, Bailey J, Thavamani A et al. Nutrition in clinical practice : official publication of the American Society for Parenteral and Enteral Nutrition (2025)
[5]
Addressing pain in people living with cystic fibrosis: Cystic fibrosis foundation evidence-informed guidelines. — Dellon EP, Allada G, Allgood SJ, Georgiopoulos AM, Goggin JL, Hadjiliadis D et al. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society (2025)
[6]
Regulatory adverse drug reaction analyses support a temporal increase in psychiatric reactions after initiation of cystic fibrosis combination modulator therapies. — O'Connor J, Nazareth D, Wat D, Southern KW, Frost F Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society (2025)
[7]
Cardiometabolic risk factors in adults with cystic fibrosis undergoing elexacaftor/tezacaftor/ivacaftor therapy. — Gramegna A, Ruscica M, Leonardi G, Macchi C, Fichtner I, Putti G et al. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society (2025)
[8]
ESPEN-ESPGHAN-ECFS guideline on nutrition care for cystic fibrosis. — Wilschanski M, Munck A, Carrion E, Cipolli M, Collins S, Colombo C et al. Clinical nutrition (Edinburgh, Scotland) (2024)
[9]
Cystic Fibrosis Foundation Evidence-Based Guideline for the Management of CRMS/CFSPID. — Green DM, Lahiri T, Raraigh KS, Ruiz F, Spano J, Antos N et al. Pediatrics (2024)
[10]
Lung ultrasound evaluation in people with cystic fibrosis: A new approach in the pulmonology outpatient clinic. — Curatola A, Corona F, Squillaci D, Saccari A, Chiaretti A, Barbi E et al. Pediatric pulmonology (2024)
[11]
Towards a Standardized Classification of the Hepatobiliary Manifestations in Cystic Fibrosis (CFHBI): A Joint ESPGHAN/NASPGHAN Position Paper. — Bodewes FAJA, Freeman AJ, Weymann A, Debray D, Scheers I, Verkade HJ et al. Journal of pediatric gastroenterology and nutrition (2024)
[12]
Calcium, Phosphorus, and Vitamin D Levels in a Series of Cystic Fibrosis Patients: A Cross-Sectional Study. — Escobedo-Monge MF, Marcos-Temprano M, Parodi-Román J, Escobedo-Monge MA, Alonso-Vicente C, Torres-Hinojal MC et al. International journal of molecular sciences (2024)
[13]
Evaluation of the efficiency of nanomicellar formulation of fat-soluble vitamins in patients with cystic fibrosis: the study protocol for a randomized controlled trial. — Soleimanzadeh M, Talebi S, Jaafari MR, Sayedi SJ, Emadzadeh M, Kianifar HR Trials (2024)
[14]
Influence of the BsmI polymorphism of the vitamin D receptor gene on the levels of vitamin D, inflammatory and oxidative stress profile in patients with cystic fibrosis supplemented with Colecalciferol megadose. — Queiroz DJM, Silva AS, da Silva Júnior CC, Paiva MP, Assis CS, Persuhn DC et al. Clinical nutrition ESPEN (2024)
[15]
Vitamin D status and variable responses to supplements depend in part on genetic factors in adults with cystic fibrosis. — Braun AT, Lai HJ, Laxova A, Biller JA, Hubertz EK, Zhao Z et al. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society (2024)
[16]
Venous thromboembolism management in people with cystic fibrosis. — Abbinanti A, Witt DM, Saunders J, Jones AE, Young DC Pediatric pulmonology (2024)
[17]
How Would We Treat Our Own Cystic Fibrosis With Lung Transplantation? — von Dossow V, Hulde N, Starke H, Schramm R Journal of cardiothoracic and vascular anesthesia (2024)
[18]
Safety of non-cuffed tunneled central venous catheters in adults with cystic fibrosis. — Dehbozorgi A, Jandali B, Turner R, Rohr A, Custer B, Young K et al. Respiratory medicine and research (2024)
[19]
Acneiform Eruption Following Elexacaftor-Tezacaftor-Ivacaftor Treatment in Patients With Cystic Fibrosis. — Okroglic L, Sohier P, Martin C, Lheure C, Franck N, Honoré I et al. JAMA dermatology (2023)
[20]
Prevalence of unmet pain and symptom management needs in adults with cystic fibrosis. — Dubin E, Lowers J, Dellon EP, Hempstead S, Faro A, Tallarico E et al. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society (2023)
[21]
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