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References
- Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA.Science. 1989; 245: 1066-1073
- Cystic fibrosis since 1938.Am J Respir Crit Care Med. 2006; 173: 475-482
- Diagnostic sweat testing: the cystic fibrosis foundation guidelines.J Pediatr. 2007; 151: 85-89
- Diagnosis of cystic fibrosis in the republic of Ireland: epidemiology and costs.Ir Med J. 2007; 100: 557-560
- A survey of newborn screening for cystic fibrosis in Europe.J Cyst Fibros. 2007; 6: 57-65
- Newborn screening for CF across the Globe—where is it worthwhile?.Int J Neonatal Screen. 2020; 6: 18
- Newborn screening for cystic fibrosis in Alberta: two years of experience.Paediatr Child Health. 2010; 15: 590-594
- 2019 Annual data report. cystic fibrosis foundation patient registry.(Available at:) (Accessed November 21, 2021)
- Comparison of the clinical manifestations of cystic fibrosis in black and white patients.J Pediatr. 1998; 132: 225-259
- Clinical sensitivity of prenatal screening for cystic fibrosis via CFTR carrier testing in a United States panethnic population.Genet Med. 2004; 6: 405-414
- Cystic fibrosis in the Ohio amish: gene frequency and founder effect.Hum Genet. 1983; 65: 94-98
- Early diagnosis of cystic fibrosis in jordanian children.J Trop Pediatr. 1992; 38: 113-115
- The impact of a national population carrier screening program on cystic fibrosis birth rate and age at diagnosis: implications for newborn screening.J Cyst Fibros. 2016; 15: 460-466
- Cystic fibrosis in Asian Indians.Arch Pediatr Adolesc Med. 1996; 150: 554-555
- Carrier frequency of F508del mutation of cystic fibrosis in Indian population.J Cyst Fibros. 2006; 5: 43-46
- Cystic fibrosis in India.Pediatr Pulmonol. 2007; 42: 1087-1094
- The estimated incidence of cystic fibrosis in Japan.J Pediatr Gastroenterol Nutr. 1997; 24: 544-547
- 2019 annual data report. the cystic fibrosis registry of Ireland.(Available at:) (Accessed November 17, 2021)
- Survival estimates in European cystic fibrosis patients and the impact of socioeconomic factors: a retrospective registry cohort study.Eur Respir J. 2021; 582002288
- Survival comparison of patients with cystic fibrosis in Canada and the United States: a population-based cohort study.Ann Intern Med. 2017; 166: 537-546
- Abnormal electrolyte composition of sweat in cystic fibrosis of the pancreas; clinical significance and relationship to the disease.Pediatrics. 1953; 12: 549-563
- Application of DNA analysis in a population-screening program for neonatal diagnosis of cystic fibrosis (CF): comparison of screening protocols.Am J Hum Genet. 1993; 52: 616-626
- Early diagnosis of cystic fibrosis through neonatal screening prevents severe malnutrition and improves long-term growth.Pediatrics. 2001; 107: 1-13
- Newborn screening for cystic fibrosis: evaluation of benefits and risks and recommendations for state newborn screening programs.MMWR Recomm Rep. 2004; 53: 1-36
- Diagnosis of cystic fibrosis: consensus guidelines from the cystic fibrosis foundation.J Pediatr. 2017; 181S: S4-S15
- Newborn screening showing decreasing incidence of cystic fibrosis.N Engl J Med. 2008; 358: 973-974
- Consistent incidence of cystic fibrosis in a long-term newborn screen population.Pediatr Pulmonol. 2008; 43: 272
- Changing incidence of cystic fibrosis in Wisconsin, USA.Pediatr Pulmonol. 2015; 50: 1065-1072
- Association between carrier screening and incidence of cystic fibrosis.JAMA. 2009; 302: 2573
- Cystic fibrosis in the year 2020: a disease with a new face.Acta Paediatr. 2020; 109: 892-899
- 1999 annual data report.Cystic Fibrosis Foundation, Bethesda, Maryland2000
- 2009 annual data report.Cystic Fibrosis Foundation, Bethesda, Maryland2010
- Relation of sweat chloride concentration to severity of lung disease in cystic fibrosis.Pediatr Pulmonol. 2004; 38: 204-209
- Modifier genetics: cystic fibrosis.Annu Rev Genomics Hum Genet. 2005; 6: 237-260
- Cystic fibrosis disease modifiers: complex genetics defines the phenotypic diversity in a monogenic disease.Annu Rev Genomics Hum Genet. 2018; 19: 201-222
- Clinical use of Ibuprofen is associated with slower FEV1 decline in children with cystic fibrosis.Am J Respir Crit Care Med. 2007; 176: 1084-1089
- Association of high-dose ibuprofen use, lung function decline, and long-term survival in children with cystic fibrosis.Ann Am Thorac Soc. 2018; 15: 485-493
- Azithromycin in patients with cystic fibrosis chronically infected with Pseudomonas aeruginosa.JAMA. 2003; 290: 1749
- The international thoracic organ transplant registry of the international society for heart and lung transplantation: thirty-fifth adult lung and heart-lung transplant report—2018; focus theme: multi-organ transplantation.J Heart Lung Transplant. 2018; 37: 1169-1183
- Heterogeneity in survival in adult patients with cystic fibrosis with FEV1 < 30% of predicted in the United States.Chest. 2017; 151: 1320-1328
- A CFTR potentiator in patients with cystic fibrosis and the G551D mutation.N Engl J Med. 2011; 365: 1663-1672
- Novel approach allows expansion of indication for cystic fibrosis drug. In: Spotlight on center for drug evaluation and research science.(Available at:) (Accessed: November 27, 2021)
- CFTR modulator theratyping: current status, gaps and future directions.J Cyst Fibros. 2019; 18: 22-34
- Lumacaftor–Ivacaftor in patients with cystic fibrosis homozygous for Phe508del CFTR.N Engl J Med. 2015; 373: 220-231
- Tezacaftor–Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del.N Engl J Med. 2017; 377: 2013-2023
- Elexacaftor–Tezacaftor–Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele.N Engl J Med. 2019; 381: 1809-1819
- Sustained benefit from ivacaftor demonstrated by combining clinical trial and cystic fibrosis patient registry data.Am J Respir Crit Care Med. 2015; 192: 836-842
- Long term clinical effectiveness of ivacaftor in people with the G551D CFTR mutation.J Cyst Fibros. 2021; 20: 213-219
- Assessment of safety and efficacy of long-term treatment with combination lumacaftor and ivacaftor therapy in patients with cystic fibrosis homozygous for the F508del-CFTR mutation (PROGRESS): a phase 3, extension study.Lancet Respir Med. 2017; 5: 107-118
- Long-term safety and efficacy of tezacaftor–ivacaftor in individuals with cystic fibrosis aged 12 years or older who are homozygous or heterozygous for Phe508del CFTR (EXTEND): an open-label extension study.Lancet Respir Med. 2021; 9: 733-746
- Cystic Fibrosis: a worldwide analysis of CFTR mutations -- correlation with incidence data and application to screening.Hum Mutat. 2002; 19: 575-606
- The demographics of adverse outcomes in cystic fibrosis.Pediatr Pulmonol. 2019; 54: S74-S83
- Disparities in mortality of hispanic patients with cystic fibrosis in the United States. A national and regional cohort study.Am J Respir Crit Care Med. 2018; 198: 1055-1063
- Pulmonary function disparities exist and persist in Hispanic patients with cystic fibrosis: a longitudinal analysis.Pediatr Pulmonol. 2017; 52: 1550-1557
- Regional variations in longitudinal pulmonary function: a comparison of Hispanic and non-Hispanic subjects with cystic fibrosis in the United States.Pediatr Pulmonol. 2019; 54: 1382-1390
- Cystic fibrosis newborn screening programs: implications of the CFTR variant spectrum in nonwhite patients.Genet Med. 2016; 19: 36-44
- Complications associated with symptomatic diagnosis in infants with cystic fibrosis.J Pediatr. 2005; 147: S37-S41
- Newborn screening for cystic fibrosis: an opportunity to improve care and outcomes.J Pediatr. 2005; 147: S2-S5
- Cystic fibrosis patients of minority race and ethnicity less likely eligible for CFTR modulators based on CFTR genotype.Pediatr Pulmonol. 2021; 56: 1496-1503
- Pseudomonas cepacia colonization among patients with cystic fibrosis.Am Rev Respir Dis. 1985; 131: 791-796
- 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: i1-i22
- The global impact of SARS-CoV-2 in 181 people with cystic fibrosis.J Cyst Fibros. 2020; 19: 868-871
- Outcomes of SARS-CoV-2 infection in patients with cystic fibrosis: a multicenter retrospective research network study.Respir Med. 2021; 188: 106606
- 2016 center specific data report.Cystic Fibrosis Foundation, Bethesda, Maryland2017
- Wasting as an independent predictor of mortality in patients with cystic fibrosis.Thorax. 2001; 56: 746-750
- Stature as a prognostic factor in cystic fibrosis survival.J Am Diet Assoc. 2001; 101: 438-442
- Stunting is an independent predictor of mortality in patients with cystic fibrosis.Clin Nutr. 2013; 32: 382-385
- CFTR modulators: impact on fertility, pregnancy, and lactation in women with cystic fibrosis.J Clin Med. 2020; 9: 2706
- Outcomes of pregnancy in women with cystic fibrosis (CF) taking CFTR modulators – an international survey.J Cyst Fibros. 2020; 19: 521-526
- Normal pancreatic function and false-negative CF newborn screen in a child born to a mother taking CFTR modulator therapy during pregnancy.J Cyst Fibros. 2021; 20: 835-836
- Renal involvement and metabolic alterations in adults patients affected by cystic fibrosis.J Transl Med. 2019; 17: 388
- Risk factors for hearing loss in patients with cystic fibrosis.J Am Acad Audiol. 2016; 27: 6-12
- Superior vena cava syndrome related to indwelling intravenous catheters in patients with cystic fibrosis.Pediatr Pulmonol. 2006; 41: 683-687
- Cystic fibrosis colorectal cancer screening consensus recommendations.Gastroenterology. 2018; 154: 736-745
- Increased augmentation index in patients with cystic fibrosis.Eur Respir J. 2009; 34: 1322-1328
- Premature vascular ageing in cystic fibrosis.Eur Respir J. 2009; 34: 1217-1218
- Is cellular senescence involved in cystic fibrosis?.Respir Res. 2019; 20: 32
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