Detalhe da pesquisa
1.
Cystic fibrosis in Europe: improved lung function and longevity - reasons for cautious optimism, but challenges remain.
Eur Respir J
; 63(3)2024 Mar.
Artigo
em Inglês
| MEDLINE | ID: mdl-38302155
2.
ABCA3-related interstitial lung disease beyond infancy.
Thorax
; 78(6): 587-595, 2023 06.
Artigo
em Inglês
| MEDLINE | ID: mdl-36808083
3.
Survival-Adjusted FEV1 and BMI Percentiles for Patients with Cystic Fibrosis before the Era of Triple CFTR Modulator Therapy in Germany.
Respiration
; 102(5): 1, 2023.
Artigo
em Inglês
| MEDLINE | ID: mdl-37062281
4.
Effects of Elexacaftor/Tezacaftor/Ivacaftor Therapy on CFTR Function in Patients with Cystic Fibrosis and One or Two F508del Alleles.
Am J Respir Crit Care Med
; 205(5): 540-549, 2022 03 01.
Artigo
em Inglês
| MEDLINE | ID: mdl-34936849
5.
Effects of Elexacaftor/Tezacaftor/Ivacaftor Therapy on Lung Clearance Index and Magnetic Resonance Imaging in Patients with Cystic Fibrosis and One or Two F508del Alleles.
Am J Respir Crit Care Med
; 206(3): 311-320, 2022 08 01.
Artigo
em Inglês
| MEDLINE | ID: mdl-35536314
6.
Survival estimates in European cystic fibrosis patients and the impact of socioeconomic factors: a retrospective registry cohort study.
Eur Respir J
; 58(3)2021 09.
Artigo
em Inglês
| MEDLINE | ID: mdl-33678607
7.
Altered relaxation times in MRI indicate bronchopulmonary dysplasia.
Thorax
; 75(2): 184-187, 2020 02.
Artigo
em Inglês
| MEDLINE | ID: mdl-31048507
8.
Effects of Lumacaftor-Ivacaftor Therapy on Cystic Fibrosis Transmembrane Conductance Regulator Function in Phe508del Homozygous Patients with Cystic Fibrosis.
Am J Respir Crit Care Med
; 197(11): 1433-1442, 2018 06 01.
Artigo
em Inglês
| MEDLINE | ID: mdl-29327948
9.
Prevalence of meconium ileus marks the severity of mutations of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene.
Genet Med
; 18(4): 333-40, 2016 Apr.
Artigo
em Inglês
| MEDLINE | ID: mdl-26087176
10.
Intestinal current measurement versus nasal potential difference measurements for diagnosis of cystic fibrosis: a case-control study.
BMC Pulm Med
; 14: 156, 2014 Oct 04.
Artigo
em Inglês
| MEDLINE | ID: mdl-25280757
11.
Effectiveness of lumacaftor/ivacaftor initiation in children with cystic fibrosis aged 2 through 5 years on disease progression: Interim results from an ongoing registry-based study.
J Cyst Fibros
; 2024 Feb 23.
Artigo
em Inglês
| MEDLINE | ID: mdl-38402082
12.
Use of elexacaftor/tezacaftor/ivacaftor leads to changes in detection frequencies of Staphylococcus aureus and Pseudomonas aeruginosa dependent on age and lung function in people with cystic fibrosis.
Int J Infect Dis
; 139: 124-131, 2024 Feb.
Artigo
em Inglês
| MEDLINE | ID: mdl-38036261
13.
A pilot study of the characterization of hepatic tissue strain in children with cystic-fibrosis-associated liver disease (CFLD) by acoustic radiation force impulse imaging.
Pediatr Radiol
; 43(5): 552-7, 2013 Mar.
Artigo
em Inglês
| MEDLINE | ID: mdl-23192669
14.
Epidemiological trends in nontuberculous mycobacterial infection among people with cystic fibrosis in Germany.
Int J Infect Dis
; 129: 32-39, 2023 Apr.
Artigo
em Inglês
| MEDLINE | ID: mdl-36736578
15.
Risk factors for forced expiratory volume in 1 s decline in European patients with cystic fibrosis: data from the European Cystic Fibrosis Society Patient Registry.
ERJ Open Res
; 9(3)2023 May.
Artigo
em Inglês
| MEDLINE | ID: mdl-37483280
16.
Corrigendum: Elexacaftor-Tezacaftor-Ivacaftor treatment reduces abdominal symptoms in cystic fibrosis-early results obtained with the CF-specific CFAbd-Score.
Front Pharmacol
; 14: 1207356, 2023.
Artigo
em Inglês
| MEDLINE | ID: mdl-37205908
17.
Impact of elexacaftor/tezacaftor/ivacaftor on lung function, nutritional status, pulmonary exacerbation frequency and sweat chloride in people with cystic fibrosis: real-world evidence from the German CF Registry.
Lancet Reg Health Eur
; 32: 100690, 2023 Sep.
Artigo
em Inglês
| MEDLINE | ID: mdl-37554663
18.
Effects of Lumacaftor/Ivacaftor on Cystic Fibrosis Disease Progression in Children 2 through 5 Years of Age Homozygous for F508del-CFTR: A Phase 2 Placebo-controlled Clinical Trial.
Ann Am Thorac Soc
; 20(8): 1144-1155, 2023 08.
Artigo
em Inglês
| MEDLINE | ID: mdl-36943405
19.
Nonsense mutations accelerate lung disease and decrease survival of cystic fibrosis children.
J Cyst Fibros
; 22(6): 1070-1079, 2023 Nov.
Artigo
em Inglês
| MEDLINE | ID: mdl-37422433
20.
Data accuracy, consistency and completeness of the national Swiss cystic fibrosis patient registry: Lessons from an ECFSPR data quality project.
J Cyst Fibros
; 2023 Nov 22.
Artigo
em Inglês
| MEDLINE | ID: mdl-37996316