Research & Literature

Changing Epidemiology of the Respiratory Bacteriology of Patients With Cystic Fibrosis.

Salsgiver EL, Fink AK, Knapp EA, et al.

Chest 2016; (149(2)):390-400 doi:10.1378/chest.15-0676.

Depression in cystic fibrosis; Implications of The International Depression/Anxiety Epidemiological Study (TIDES) in cystic fibrosis.

Duff AJ

Paediatric respiratory reviews 2015; (16 Suppl 1()):2-5.

Lumacaftor alone and combined with ivacaftor: preclinical and clinical trial experience of F508del CFTR correction.

Brewington JJ, McPhail GL, Clancy JP

Expert review of respiratory medicine 2016; (10(1)):5-17 doi:10.1586/17476348.2016.1122527.

Bacterial colonization status of cystic fibrosis children's toothbrushes: A pilot study.

Genevois A, Roques C, Segonds C, et al.

Archives de pediatrie : organe officiel de la Societe francaise de pediatrie 2015; (22(12)):1240-6.

Development and Validation of a PCR Assay To Detect the Prairie Epidemic Strain of Pseudomonas aeruginosa from Patients with Cystic Fibrosis.

Workentine M, Poonja A, Waddell B, et al.

Journal of clinical microbiology 2016; (54(2)):489-91 doi:10.1128/JCM.02603-15.

The psychological burden of cystic fibrosis.

Quittner AL, Saez-Flores E, Barton JD

Current opinion in pulmonary medicine 2016; (22(2)):187-91 doi:10.1097/MCP.0000000000000244.

Hydrator Therapies for Chronic Bronchitis. Lessons from Cystic Fibrosis.

Bennett WD, Henderson AG, Donaldson SH

Annals of the American Thoracic Society 2016; (13 Suppl 2()):S186-90 doi:10.1513/AnnalsATS.201509-652KV.

They know it's safe - they know what to expect from that face: perceptions towards a cognitive-behavioural counselling programme among caregivers of children with cystic fibrosis.

Moola FJ, Henry LA, Huynh E, et al.

Journal of clinical nursing 2017; (26(19-20)):2932-2943 doi:10.1111/jocn.13622.

BMI fails to identify poor nutritional status in stunted children with CF.

Konstan MW, Pasta DJ, Wagener JS, et al.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society 2017; (16(1)):158-160 doi:10.1016/j.jcf.2016.11.005.

Diagnosis of Cystic Fibrosis: Consensus Guidelines from the Cystic Fibrosis Foundation.

Farrell PM, White TB, Ren CL, et al.

The Journal of pediatrics 2017; (181S()):S4-S15.e1 doi:10.1016/j.jpeds.2016.09.064.

Diagnosis of Cystic Fibrosis in Nonscreened Populations.

Sosnay PR, White TB, Farrell PM, et al.

The Journal of pediatrics 2017; (181S()):S52-S57.e2 doi:10.1016/j.jpeds.2016.09.068.

[Adolescents with cystic fibrosis: the approach to transition from paediatric to adult care].

Murris-Espin M, Le Borgne A, Vignal G, et al.

Archives de pediatrie : organe officiel de la Societe francaise de pediatrie 2016; (23(12S)):12S54-12S60 doi:10.1016/S0929-693X(17)30063-5.

Population Dynamics of Staphylococcus aureus in Cystic Fibrosis Patients To Determine Transmission Events by Use of Whole-Genome Sequencing.

Ankrum A, Hall BG

Journal of clinical microbiology 2017; (55(7)):2143-2152 doi:10.1128/JCM.00164-17.

Current Concepts of Transition of Care in Cystic Fibrosis.

Patel A, Dowell M, Giles BL

Pediatric annals 2017; (46(5)):e188-e192 doi:10.3928/19382359-20170425-02.

The diagnosis of cystic fibrosis.

De Boeck K, Vermeulen F, Dupont L

Presse medicale (Paris, France : 1983) 2017; (46(6 Pt 2)):e97-e108 doi:10.1016/j.lpm.2017.04.010.

Congenital bilateral absence of the vas deferens as an atypical form of cystic fibrosis: reproductive implications and genetic counseling.

de Souza DAS, Faucz FR, Pereira-Ferrari L, et al.

Andrology 2018; (6(1)):127-135 doi:10.1111/andr.12450.

Revisiting sweat chloride test results based on recent guidelines for diagnosis of cystic fibrosis.

Pagaduan JV, Ali M, Dowlin M, et al.

Practical laboratory medicine 2018; (10()):34-37 doi:10.1016/j.plabm.2018.01.001.

Intra-individual biological variation in sweat chloride concentrations in CF, CFTR dysfunction, and healthy pediatric subjects.

Cirilli N, Raia V, Rocco I, et al.

Pediatric pulmonology 2018; (53(6)):728-734 doi:10.1002/ppul.23992.

Acute Appendicitis Masquerading Distal Intestinal Obstruction Syndrome in Adult Cystic Fibrosis.

Nanavati SM, Patel H, Melki G, et al.

Case reports in gastrointestinal medicine 2018; (2018()):8475060 doi:10.1155/2018/8475060.

[Patients with cystic fibrosis become adults : Treatment hopes and disappointments].

Eschenhagen P, Schwarz C

Der Internist 2019; (60(1)):98-108 doi:10.1007/s00108-018-0536-9.

Inhaled medications in cystic fibrosis beyond antibiotics.

Sepe A, Villella VR, Cimbalo C, et al.

Minerva pediatrica 2019; (71(4)):371-375 doi:10.23736/S0026-4946.19.05509-9.

Review of Cystic Fibrosis.

Goetz D, Ren CL

Pediatric annals 2019; (48(4)):e154-e161 doi:10.3928/19382359-20190327-01.

Cystic fibrosis program characteristics associated with adoption of 2013 infection prevention and control recommendations.

Stoudemire W, Jiang X, Zhou JJ, et al.

American journal of infection control 2019; (47(9)):1090-1095 doi:10.1016/j.ajic.2019.03.015.

Acute Recurrent and Chronic Pancreatitis as Initial Manifestations of Cystic Fibrosis and Cystic Fibrosis Transmembrane Conductance Regulator-Related Disorders.

Baldwin C, Zerofsky M, Sathe M, et al.

Pancreas 2019; (48(7)):888-893 doi:10.1097/MPA.0000000000001350.

Caregiver burden in children with cystic fibrosis and primary ciliary dyskinesia.

Keniş Coşkun Ö, Gençer Atalay K, Erdem E, et al.

Pediatric pulmonology 2019; (54(12)):1936-1940 doi:10.1002/ppul.24503.

Uncommon clinical presentation of cystic fibrosis in a patient homozygous for a rare CFTR mutation: a case report.

Jaworska J, Marach-Mocarska A, Sands D

BMC pediatrics 2020; (20(1)):90 doi:10.1186/s12887-020-1980-y.

Genetic diagnosis in practice: From cystic fibrosis to CFTR-related disorders.

Pagin A, Sermet-Gaudelus I, Burgel PR

Archives de pediatrie : organe officiel de la Societe francaise de pediatrie 2020; (27 Suppl 1()):eS25-eS29 doi:10.1016/S0929-693X(20)30047-6.

Who's at The Door? - Surface Contamination of Door Frames in a Single-Bedded In-Patient Adult Cystic Fibrosis (CF) Unit.

Furukawa M, McCaughan J, Stirling J, et al.

The Ulster medical journal 2020; (89(1)):17-20.

Emerging Cystic Fibrosis Transmembrane Conductance Regulator Modulators as New Drugs for Cystic Fibrosis: A Portrait of in Vitro Pharmacology and Clinical Translation.

Ghelani DP, Schneider-Futschik EK

ACS pharmacology & translational science 2020; (3(1)):4-10 doi:10.1021/acsptsci.9b00060.

Elexacaftor-Tezacaftor-Ivacaftor: The First Triple-Combination Cystic Fibrosis Transmembrane Conductance Regulator Modulating Therapy.

Ridley K, Condren M

The journal of pediatric pharmacology and therapeutics : JPPT : the official journal of PPAG 2020; (25(3)):192-197 doi:10.5863/1551-6776-25.3.192.

The preclinical discovery and development of the combination of ivacaftor + tezacaftor used to treat cystic fibrosis.

Guerra L, Favia M, Di Gioia S, et al.

Expert opinion on drug discovery 2020; (15(8)):873-891 doi:10.1080/17460441.2020.1750592.

Dried Blood Spot-Based Metabolomic Profiling in Adults with Cystic Fibrosis.

Al-Qahtani W, Abdel Jabar M, Masood A, et al.

Journal of proteome research 2020; (19(6)):2346-2357 doi:10.1021/acs.jproteome.0c00031.

Patients eligible for modulator drugs: Data from cystic fibrosis registry of Turkey.

Çobanoğlu N, Özçelik U, Çakır E, et al.

Pediatric pulmonology 2020; (55(9)):2302-2306 doi:10.1002/ppul.24854.

Pro con debates in clinical medicine infection prevention and control in cystic fibrosis: One size fits all? The argument in favour.

Haggie S, Fitzgerald DA

Paediatric respiratory reviews 2020; (36()):97-99 doi:10.1016/j.prrv.2020.06.008.

Pharmacological and Genomic Approaches in Management of Cystic Fibrosis.

Qadir MI, Yameen IA

Critical reviews in eukaryotic gene expression 2020; (30(3)):191-197 doi:10.1615/CritRevEukaryotGeneExpr.2020030845.

Decreased survival in cystic fibrosis patients with a positive screen for depression.

Schechter MS, Ostrenga JS, Fink AK, et al.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society 2021; (20(1)):120-126 doi:10.1016/j.jcf.2020.07.020.

Intestinal current measurement and nasal potential difference to make a diagnosis of cases with inconclusive CFTR genetics and sweat test.

Minso R, Schulz A, Dopfer C, et al.

BMJ open respiratory research 2020; (7(1)) doi:10.1136/bmjresp-2020-000736.

Newborn Screening for CF across the Globe-Where Is It Worthwhile?

Scotet V, Gutierrez H, Farrell PM

International journal of neonatal screening 2020; (6(1)):18.

Inconclusive Diagnosis after Newborn Screening for Cystic Fibrosis.

Munck A

International journal of neonatal screening 2020; (6(1)):19.

The first five-year evaluation of cystic fibrosis neonatal screening program in São Paulo State, Brazil.

Maciel LMZ, Magalhães PKR, Ciampo IRLD, et al.

Cadernos de saude publica 2020; (36(10)):e00049719 doi:10.1590/0102-311X00049719.

[Evidence-based treatment of cystic fibrosis].

Ringshausen FC, Hellmuth T, Dittrich AM

Der Internist 2020; (61(12)):1212-1229 doi:10.1007/s00108-020-00896-9.

The Effect of Self-Efficacy, Social Support and Quality of Life on Readiness for Transition to Adult Care Among Adolescents with Cystic Fibrosis in Turkey.

Torun T, Çavuşoğlu H, Doğru D, et al.

Journal of pediatric nursing 2021; (57()):e79-e84 doi:10.1016/j.pedn.2020.11.013.

A current review of the safety of cystic fibrosis transmembrane conductance regulator modulators.

Gavioli EM, Guardado N, Haniff F, et al.

Journal of clinical pharmacy and therapeutics 2021; (46(2)):286-294 doi:10.1111/jcpt.13329.

Rescue of multiple class II CFTR mutations by elexacaftor+tezacaftor+ivacaftor mediated in part by the dual activities of elexacaftor as both corrector and potentiator.

Laselva O, Bartlett C, Gunawardena TNA, et al.

The European respiratory journal 2021; (57(6)) doi:10.1183/13993003.02774-2020.

Is CFTR an exchanger?: Regulation of HCO3 -Transport and extracellular pH by CFTR.

Massey MK, Reiterman MJ, Mourad J, Luckie DB

Biochemistry and biophysics reports 2021; (25()):100863 doi:10.1016/j.bbrep.2020.100863.

Screening of depression and anxiety in adolescents with cystic fibrosis and caregivers in Turkey by PHQ-9 and GAD-7 questionnaires.

Mursaloğlu HH, Yılmaz Yeğit C, Ergenekon AP, et al.

Pediatric pulmonology 2021; (56(6)):1514-1520 doi:10.1002/ppul.25295.

Transmission, adaptation and geographical spread of the Pseudomonas aeruginosa Liverpool epidemic strain.

Moore MP, Lamont IL, Williams D, et al.

Microbial genomics 2021; (7(3)) doi:10.1099/mgen.0.000511.

Partnership Enhancement Program: Piloting a Communication Training Program for Cystic Fibrosis Care Teams.

George C, Raymond KF, Collins L, et al.

Journal of patient experience 2021; (8()):23743735211014049 doi:10.1177/23743735211014049.

Immediate effect of inhalation therapy combined with oscillatory positive expiratory pressure on the respiratory system of children with cystic fibrosis.

Gonçalves Wamosy RM, Castilho T, Almeida ACDS, et al.

International journal of clinical practice 2021; (75(10)):e14659 doi:10.1111/ijcp.14659.

CFTR modulator use in post lung transplant recipients.

Benninger LA, Trillo C, Lascano J

The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation 2021; (40(12)):1498-1501 doi:10.1016/j.healun.2021.08.009.

Outcomes of early repeat sweat testing in infants with cystic fibrosis transmembrane conductance regulator-related metabolic syndrome/CF screen-positive, inconclusive diagnosis.

Terlizzi V, Claut L, Colombo C, et al.

Pediatric pulmonology 2021; (56(12)):3785-3791 doi:10.1002/ppul.25683.

Quality of home spirometry performance amongst adults with cystic fibrosis.

Bell JM, Sivam S, Dentice RL, et al.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society 2022; (21(1)):84-87 doi:10.1016/j.jcf.2021.10.012.

Effect of elexacaftor-tezacaftor-ivacaftor on body weight and metabolic parameters in adults with cystic fibrosis.

Petersen MC, Begnel L, Wallendorf M, Litvin M

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society 2022; (21(2)):265-271 doi:10.1016/j.jcf.2021.11.012.

CF and male health: Sexual and reproductive health, hypogonadism, and fertility.

Naz Khan F, Mason K, Roe AH, Tangpricha V

Journal of clinical & translational endocrinology 2022; (27()):100288 doi:10.1016/j.jcte.2021.100288.

Cystic fibrosis transmembrane conductance regulator-related metabolic syndrome/cystic fibrosis screen positive, inconclusive diagnosis (CRMS/CFSPID).

Sinha A, Southern KW

Breathe (Sheffield, England) 2021; (17(3)):210088 doi:10.1183/20734735.0088-2021.

Precision Medicine Based on CFTR Genotype for People with Cystic Fibrosis.

Haq I, Almulhem M, Soars S, et al.

Pharmacogenomics and personalized medicine 2022; (15()):91-104 doi:10.2147/PGPM.S245603.

Moving up: Healthcare transition experiences of adolescents and young adults with cystic fibrosis.

South K, George M, Sadeghi H, et al.

Journal of pediatric nursing 2022; (65()):116-123 doi:10.1016/j.pedn.2022.03.007.

Novel CFTR modulator combinations maximise rescue of G85E and N1303K in rectal organoids.

Ensinck MM, De Keersmaecker L, Ramalho AS, et al.

ERJ open research 2022; (8(2)) doi:10.1183/23120541.00716-2021.

Analyzing the use and impact of elexacaftor/tezacaftor/ivacaftor on total cost of care and other health care resource utilization in a commercially insured population.

Smith S, Borchardt M

Journal of managed care & specialty pharmacy 2022; (28(7)):721-731 doi:10.18553/jmcp.2022.28.7.721.

Behavioral Health Screening in Military Cystic Fibrosis Centers: A Survey.

Rutledge C, Gould C, Lee PC, et al.

Military medicine 2022; doi:10.1093/milmed/usac161.

False-Positive and False-Negative Sweat Tests: Systematic Review of the Evidence.

Guglani L, Stabel D, Weiner DJ

Pediatric allergy, immunology, and pulmonology 2015; (28(4)):198-211 doi:10.1089/ped.2015.0552.

Infection control in cystic fibrosis: evolving perspectives and challenges.

Jones AM

Current opinion in pulmonary medicine 2022; (28(6)):571-576 doi:10.1097/MCP.0000000000000918.

ECFS standards of care on CFTR-related disorders: Diagnostic criteria of CFTR dysfunction.

Sermet-Gaudelus I, Girodon E, Vermeulen F, et al.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society 2022; (21(6)):922-936 doi:10.1016/j.jcf.2022.09.005.

ECFS standards of care on CFTR-related disorders: Updated diagnostic criteria.

Castellani C, De Boeck K, De Wachter E, et al.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society 2022; (21(6)):908-921 doi:10.1016/j.jcf.2022.09.011.

Molecular structures reveal synergistic rescue of Δ508 CFTR by Trikafta modulators.

Fiedorczuk K, Chen J

Science (New York, N.Y.) 2022; (378(6617)):284-290 doi:10.1126/science.ade2216.

Unified Airway-Cystic Fibrosis.

Cho DY, Grayson JW, Woodworth BA

Otolaryngologic clinics of North America 2023; (56(1)):125-136 doi:10.1016/j.otc.2022.09.009.

Non-Modulator Therapies: Developing a Therapy for Every Cystic Fibrosis Patient.

Egan ME

Clinics in chest medicine 2022; (43(4)):717-725 doi:10.1016/j.ccm.2022.06.011.

Transitions of Care in Cystic Fibrosis.

DeFilippo EMM, Talwalkar JS, Harris ZM, et al.

Clinics in chest medicine 2022; (43(4)):757-771 doi:10.1016/j.ccm.2022.06.016.

Screening by high-throughput sequencing for pathogenic variants in cystic fibrosis: Benefit of introducing personalized therapies.

de Melo ACV, de Souza KSC, da Silva HPV, et al.

Journal of cellular and molecular medicine 2022; (26(23)):5943-5947 doi:10.1111/jcmm.17605.

Successful pregnancy in a cystic fibrosis patient with a severe impairment of lung function receiving Elexacaftor-Tezacaftor-Ivacaftor.

Balmpouzis Z, Faure van Rossum A, Baud D, et al.

Respiratory medicine case reports 2022; (40()):101776 doi:10.1016/j.rmcr.2022.101776.

Clinical efficacy of elexacaftor-tezacaftor-ivacaftor in an adolescent with homozygous G85E cystic fibrosis.

Stekolchik E, Saul D, Chidekel A

Respiratory medicine case reports 2022; (40()):101775 doi:10.1016/j.rmcr.2022.101775.

A Cross-Sectional Analysis of Paternal Intimacy Problems, Stress Levels, and Satisfaction from Families with Children Born with Mucoviscidosis.

Popa ZL, Margan MM, Bernad E, et al.

International journal of environmental research and public health 2022; (19(22)) doi:10.3390/ijerph192215055.

High rates of anxiety detected in mothers of children with inconclusive cystic fibrosis screening results.

Ginsburg DK, Salinas DB, Cosanella TM, et al.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society 2023; (22(3)):420-426 doi:10.1016/j.jcf.2022.12.002.

Comparison of mental health in individuals with primary ciliary dyskinesia, cystic fibrosis, and parent caregivers.

Graziano S, Ullmann N, Rusciano R, et al.

Respiratory medicine 2023; (207()):107095 doi:10.1016/j.rmed.2022.107095.

The role of CFTR channel in female infertility.

Habibullah MM

Human fertility (Cambridge, England) 2023; (26(5)):1228-1237 doi:10.1080/14647273.2022.2161427.

Diagnostic agreement among experts assessing adults presenting with possible cystic fibrosis: need for improvement and implications for patient care.

Franciosi AN, Tanzler A, Goodwin J, et al.

ERJ open research 2022; (8(4)) doi:10.1183/23120541.00227-2022.

Current and Future Therapeutic Approaches of Exocrine Pancreatic Insufficiency in Children with Cystic Fibrosis in the Era of Personalized Medicine.

Ritivoiu ME, Drăgoi CM, Matei D, et al.

Pharmaceutics 2023; (15(1)) doi:10.3390/pharmaceutics15010162.

A Multicentre Italian Study on the Psychological Impact of an Inconclusive Cystic Fibrosis Diagnosis after Positive Neonatal Screening.

Tosco A, Marino D, Polizzi S, et al.

Children (Basel, Switzerland) 2023; (10(2)) doi:10.3390/children10020177.

Use of elexacaftor/tezacaftor/ivacaftor combination in pregnancy.

Cimino G, Sorrenti S, Murciano M, et al.

Archives of gynecology and obstetrics 2024; (309(1)):9-15 doi:10.1007/s00404-023-06962-5.

Outcomes of children with cystic fibrosis screen positive, inconclusive diagnosis/CFTR related metabolic syndrome.

Gunnett MA, Baker E, Mims C, et al.

Frontiers in pediatrics 2023; (11()):1127659 doi:10.3389/fped.2023.1127659.

Real-life impact of highly effective CFTR modulator therapy in children with cystic fibrosis.

Olivier M, Kavvalou A, Welsner M, et al.

Frontiers in pharmacology 2023; (14()):1176815 doi:10.3389/fphar.2023.1176815.

Patient Attitudes Regarding Health Care Clinician Communication in Adolescents With Cystic Fibrosis.

Woolley JC, Edmondson SN, Sathe MN, Rodriguez Francis JK

Journal of pediatric gastroenterology and nutrition 2023; (77(2)):e23-e28 doi:10.1097/MPG.0000000000003844.

Effects of elexacaftor / tezacaftor / ivacaftor triple combination therapy on glycaemic control and body composition in patients with cystic fibrosis-related diabetes.

Grancini V, Gramegna A, Zazzeron L, et al.

Diabetes & metabolism 2023; (49(5)):101466 doi:10.1016/j.diabet.2023.101466.

Children with cystic fibrosis are still receiving inconclusive diagnosis despite undergoing newborn screening.

Loukou I, Moustaki M, Douros K

Acta paediatrica (Oslo, Norway : 1992) 2023; (112(10)):2039-2044 doi:10.1111/apa.16949.

Understanding and addressing the needs of people with cystic fibrosis in the era of CFTR modulator therapy.

Hisert KB, Birket SE, Clancy JP, et al.

The Lancet. Respiratory medicine 2023; (11(10)):916-931 doi:10.1016/S2213-2600(23)00324-7.

Advances in the Cystic Fibrosis Drug Development Pipeline.

Esposito C, Kamper M, Trentacoste J, et al.

Life (Basel, Switzerland) 2023; (13(9)) doi:10.3390/life13091835.

Standards for the care of people with cystic fibrosis (CF): A timely and accurate diagnosis.

Castellani C, Simmonds NJ, Barben J, et al.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society 2023; (22(6)):963-968 doi:10.1016/j.jcf.2023.09.008.

New Tic Disorder in a Child With Cystic Fibrosis Treated With Elexacaftor/Tezacaftor/Ivacaftor.

Duehlmeyer SR, Elson EC, Oermann CM

The journal of pediatric pharmacology and therapeutics : JPPT : the official journal of PPAG 2024; (29(1)):82-84 doi:10.5863/1551-6776-29.1.82.

Year in review 2023 - Back to the future.

Cristiani L, Fernandes FF

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society 2024; (23(2)):203-207 doi:10.1016/j.jcf.2024.02.007.

CFTR function is impaired in a subset of patients with pancreatitis carrying rare CFTR variants.

Angyal D, Kleinfelder K, Ciciriello F, et al.

Pancreatology : official journal of the International Association of Pancreatology (IAP) ... [et al.] 2024; (24(3)):394-403 doi:10.1016/j.pan.2024.03.005.

ECFS standards of care on CFTR-related disorders: Identification and care of the disorders.

Simmonds NJ, Southern KW, De Wachter E, et al.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society 2024; (23(4)):590-602 doi:10.1016/j.jcf.2024.03.008.

Persistence and evolution of Pseudomonas aeruginosa following initiation of highly effective modulator therapy in cystic fibrosis.

Armbruster CR, Hilliam YK, Zemke AC, et al.

mBio 2024; (15(5)):e0051924 doi:10.1128/mbio.00519-24.

Cystic Fibrosis Foundation Evidence-Based Guideline for the Management of CRMS/CFSPID.

Green DM, Lahiri T, Raraigh KS, et al.

Pediatrics 2024; (153(5)) doi:10.1542/peds.2023-064657.

[Cystic fibrosis in childhood and adulthood].

Syunyaeva Z, Mall MA, Stahl M

Innere Medizin (Heidelberg, Germany) 2024; (65(6)):538-544 doi:10.1007/s00108-024-01717-z.

Ocular Changes in Cystic Fibrosis: A Review.

Liberski S, Confalonieri F, Cofta S, et al.

International journal of molecular sciences 2024; (25(12)) doi:10.3390/ijms25126692.

Telemedicine and home spirometry in cystic fibrosis: A prospective multicenter study.

Medbo J, Imberg H, Hansen C, et al.

Pediatric pulmonology 2024; (59(11)):2967-2975 doi:10.1002/ppul.27166.

Characteristics of individuals with cystic fibrosis in the United States ineligible for ivacaftor and elexacaftor/tezacaftor/ivacaftor.

Sanders DB, Mayer-Hamblett N, Rosenfeld M, et al.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society 2025; (24(2)):255-262 doi:10.1016/j.jcf.2024.07.015.

Impact of highly effective modulator therapy on gastrointestinal symptoms and features in people with cystic fibrosis.

Cecchetti M, Scarallo L, Lionetti P, et al.

Paediatric respiratory reviews 2025; (54()):70-75 doi:10.1016/j.prrv.2024.07.004.

The Effectiveness of a Parent Empowerment Intervention for Caregivers of Children with Cystic Fibrosis: A Randomized Controlled Trial.

Donmez H, Tas Arslan F

Comprehensive child and adolescent nursing 2024; (47(4)):267-283 doi:10.1080/24694193.2024.2411986.

Phenotypic Evaluation of Rare Cystic Fibrosis Transmembrane Conductance Regulator Mutation Combinations in People with Cystic Fibrosis in Queensland, Australia.

Evans IES, Wood M, Moore V, Reid DW

Journal of clinical medicine 2024; (13(20)) doi:10.3390/jcm13206210.

Case Report: A delicate equilibrium of exocrine pancreatic recovery and hepatotoxicity with elexacaftor/tezacaftor/ivacaftor therapy in a pediatric patient with cystic fibrosis.

Coughlin MP, Sankararaman S, Roesch EA, et al.

Frontiers in pediatrics 2024; (12()):1457517 doi:10.3389/fped.2024.1457517.

Evaluating CRMS/CFSPID phenotypes and outcomes: A retrospective study from a large UK cystic fibrosis centre.

Mansfield A, Hine C, Nagakumar P, et al.

Heliyon 2024; (10(21)):e39935 doi:10.1016/j.heliyon.2024.e39935.

Discovery and Development of CFTR Modulators for the Treatment of Cystic Fibrosis.

Wang X, Tse C, Singh A

Journal of medicinal chemistry 2025; (68(3)):2255-2300 doi:10.1021/acs.jmedchem.4c02547.

Cystic fibrosis year in review 2024.

Alameeri A, Yavuz BC, Lucca F, et al.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society 2025; (24(2)):218-223 doi:10.1016/j.jcf.2025.02.012.

The Feasibility and Validity of Home Spirometry for People with Cystic Fibrosis: Is It Comparable to Spirometry in the Clinic?

Sopiadou A, Gioulvanidou M, Kogias C, et al.

Children (Basel, Switzerland) 2025; (12(3)) doi:10.3390/children12030277.

Unmasking Cystic Fibrosis in Adulthood, a Case Report.

Mohtadi M, Habib R, Rezkalla A, et al.

Journal of investigative medicine high impact case reports 2025; (13()):23247096251334248 doi:10.1177/23247096251334248.

Remote monitoring of cystic fibrosis lung disease in children and young adults.

Svedberg M, Michelsen J, Roberts E, et al.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society 2025; (24(4)):704-709 doi:10.1016/j.jcf.2025.03.670.

Australasian guideline for the performance of sweat chloride testing 3rd edition: to support cystic fibrosis screening, diagnosis and monitoring.

Massie J, McWhinney A, Greed L, et al.

Clinical chemistry and laboratory medicine 2025; (63(10)):1956-1964 doi:10.1515/cclm-2025-0433.

Impact of CFTR Modulators on Longitudinal Cystic Fibrosis Survival and Mortality: Review and Secondary Analysis.

Rubin JL, McKinnon C, Pedra GG, et al.

Pulmonary therapy 2025; (11(3)):365-386 doi:10.1007/s41030-025-00303-4.

Condition-dependent effects of Elexacaftor/Tezacaftor/Ivacaftor (Trikafta) on Aspergillus fumigatus growth.

Biquand E, Khau S, Fouquenet D, et al.

Microbiology spectrum 2025; (13(9)):e0227524 doi:10.1128/spectrum.02275-24.

Transitioning care in cystic fibrosis: a comprehensive review of reviews of clinical and psychosocial outcomes.

Alzayed A

Frontiers in pediatrics 2025; (13()):1643434 doi:10.3389/fped.2025.1643434.

Transforming Care Models in Cystic Fibrosis: A Review.

Diener BL, Berdella M, DeCelie-Germana J, et al.

Healthcare (Basel, Switzerland) 2025; (13(23)) doi:10.3390/healthcare13233022.

Case Report: Pitfalls in CF screening - targeted variant analysis can cause misleading results and therapy recommendations.

Karnstedt M, Ahting S, Behrendt S, et al.

Frontiers in genetics 2025; (16()):1693573 doi:10.3389/fgene.2025.1693573.

Developing a primer for communicating about penetrance, expressivity, and genotype-phenotype correlation of R117H and the poly-T tract in CFTR.

Raraigh KS, Balcom JR, Langfelder-Schwind E

Journal of genetic counseling 2025; (34(6)):e70151 doi:10.1002/jgc4.70151.

Divergent neurobehavioral effects of CFTR modulators elexacaftor and ivacaftor in mice.

Ge Q, Fagan A, Lu H, et al.

Acta psychologica 2026; (263()):106210 doi:10.1016/j.actpsy.2026.106210.

Therapeutic hopes for CFTR-related recurrent pancreatitis: a case-series.

Gohy S, Le A, Sermet-Gaudelus I, et al.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society 2026; doi:10.1016/j.jcf.2026.02.008.

Estimation of care costs for individuals with cystic fibrosis at a referral center: cohort study, Salvador, 2005-2022.

Costa JCB, Sorte NCAB, D'Souza EL

Epidemiologia e servicos de saude : revista do Sistema Unico de Saude do Brasil 2026; (35()):e20250072 doi:10.1590/S2237-96222026v35e20250072.en.

MLPA-Detected CFTR Exon Deletions in Children With Inconclusive CFTR Sequencing: A Single-Center Experience From Türkiye.

Eryılmaz Polat S, Yıldırım Ç, Yetişgin H, et al.

Pediatric pulmonology 2026; (61(3)):e71556 doi:10.1002/ppul.71556.