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Lung Function Decline in Cystic Fibrosis: Impact of Data Availability and Modeling Strategies on Clinical Interpretations.
Szczesniak R, Andrinopoulou ER, Su W, Afonso PM, Burgel PR, Cromwell E, Gecili E, Ghulam E, Goss CH, Mayer-Hamblett N, Keogh RH, Liou TG, Marshall B, Morgan WJ, Ostrenga JS, Pasta DJ, Stanojevic S, Wainwright C, Zhou GC, Fernandez G, Fink AK, Schechter MS. Szczesniak R, et al. Among authors: pasta dj. Ann Am Thorac Soc. 2023 Jul;20(7):958-968. doi: 10.1513/AnnalsATS.202209-829OC. Ann Am Thorac Soc. 2023. PMID: 36884219
Marginal models consistently estimated less rapid lung function decline than mixed-effects models across scenarios, except for short-term follow-up (both were 1.4). Rate of decline estimates from nonlinear models diverged by age 30. ...Conclusions: Differences were as high …
Marginal models consistently estimated less rapid lung function decline than mixed-effects models across scenarios, except for short-term …
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.
Flume PA, Biner RF, Downey DG, Brown C, Jain M, Fischer R, De Boeck K, Sawicki GS, Chang P, Paz-Diaz H, Rubin JL, Yang Y, Hu X, Pasta DJ, Millar SJ, Campbell D, Wang X, Ahluwalia N, Owen CA, Wainwright CE; VX14-661-110 study group. Flume PA, et al. Among authors: pasta dj. Lancet Respir Med. 2021 Jul;9(7):733-746. doi: 10.1016/S2213-2600(20)30510-5. Epub 2021 Feb 10. Lancet Respir Med. 2021. PMID: 33581080
Here, we present results of study 661-110 (EXTEND), a 96-week open-label extension study that assessed long-term safety, tolerability, and efficacy of tezacaftor-ivacaftor in participants aged 12 years or older with cystic fibrosis who were homozygous or heterozygous for t …
Here, we present results of study 661-110 (EXTEND), a 96-week open-label extension study that assessed long-term safety, tolerability …
Predicting Survival in Patients With Pulmonary Arterial Hypertension: The REVEAL Risk Score Calculator 2.0 and Comparison With ESC/ERS-Based Risk Assessment Strategies.
Benza RL, Gomberg-Maitland M, Elliott CG, Farber HW, Foreman AJ, Frost AE, McGoon MD, Pasta DJ, Selej M, Burger CD, Frantz RP. Benza RL, et al. Among authors: pasta dj. Chest. 2019 Aug;156(2):323-337. doi: 10.1016/j.chest.2019.02.004. Epub 2019 Feb 14. Chest. 2019. PMID: 30772387 Free article.
This analysis describes an update of the Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) risk calculator (REVEAL 2.0) and compares it with recently published European Society of Cardiology/Respiratory Society guideline-derived risk assessment strat …
This analysis describes an update of the Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) risk calculator (RE …
Association of High-Dose Ibuprofen Use, Lung Function Decline, and Long-Term Survival in Children with Cystic Fibrosis.
Konstan MW, VanDevanter DR, Sawicki GS, Pasta DJ, Foreman AJ, Neiman EA, Morgan WJ. Konstan MW, et al. Among authors: pasta dj. Ann Am Thorac Soc. 2018 Apr;15(4):485-493. doi: 10.1513/AnnalsATS.201706-486OC. Ann Am Thorac Soc. 2018. PMID: 29313709
CONCLUSIONS: In a propensity-score matched cohort study of children with cystic fibrosis, we observed an association between high-dose ibuprofen use and both slower lung function decline and improved long-term survival. These results are consistent with the hypothesis that …
CONCLUSIONS: In a propensity-score matched cohort study of children with cystic fibrosis, we observed an association between high-dose ibupr …
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.
Konstan MW, McKone EF, Moss RB, Marigowda G, Tian S, Waltz D, Huang X, Lubarsky B, Rubin J, Millar SJ, Pasta DJ, Mayer-Hamblett N, Goss CH, Morgan W, Sawicki GS. Konstan MW, et al. Among authors: pasta dj. Lancet Respir Med. 2017 Feb;5(2):107-118. doi: 10.1016/S2213-2600(16)30427-1. Epub 2016 Dec 21. Lancet Respir Med. 2017. PMID: 28011037 Clinical Trial.
BACKGROUND: The 24-week safety and efficacy of lumacaftor/ivacaftor combination therapy was shown in two randomised controlled trials (RCTs)-TRAFFIC and TRANSPORT-in patients with cystic fibrosis who were aged 12 years or older and homozygous for the F508del-CFTR mutation. We aim …
BACKGROUND: The 24-week safety and efficacy of lumacaftor/ivacaftor combination therapy was shown in two randomised controlled trials (RCTs) …
Sustained Benefit from ivacaftor demonstrated by combining clinical trial and cystic fibrosis patient registry data.
Sawicki GS, McKone EF, Pasta DJ, Millar SJ, Wagener JS, Johnson CA, Konstan MW. Sawicki GS, et al. Among authors: pasta dj. Am J Respir Crit Care Med. 2015 Oct 1;192(7):836-42. doi: 10.1164/rccm.201503-0578OC. Am J Respir Crit Care Med. 2015. PMID: 26132840
However, whether these improvements reflect a change in disease trajectory cannot be determined without longer-term analyses with an appropriate comparator population. OBJECTIVES: To examine, over a 3-year period, whether ivacaftor therapy affects pulmonary function and nu …
However, whether these improvements reflect a change in disease trajectory cannot be determined without longer-term analyses with an …
Long-term effects of pregnancy and motherhood on disease outcomes of women with cystic fibrosis.
Schechter MS, Quittner AL, Konstan MW, Millar SJ, Pasta DJ, McMullen A; Scientific Advisory Group; Investigators and Coordinators of Epidemiologic Study of Cystic Fibrosis. Schechter MS, et al. Among authors: pasta dj. Ann Am Thorac Soc. 2013 Jun;10(3):213-9. doi: 10.1513/AnnalsATS.201211-108OC. Ann Am Thorac Soc. 2013. PMID: 23802817
RATIONALE: Studies of pregnancy in cystic fibrosis (CF) have shown no short-term harmful effects, but there are no long-term studies on the impact of motherhood. OBJECTIVES: This study sought to evaluate longer-term physiologic and functional outcomes in wome …
RATIONALE: Studies of pregnancy in cystic fibrosis (CF) have shown no short-term harmful effects, but there are no long-term s …
Pulmonary exacerbations in cystic fibrosis: young children with characteristic signs and symptoms.
Regelmann WE, Schechter MS, Wagener JS, Morgan WJ, Pasta DJ, Elkin EP, Konstan MW; Investigators of the Epidemiologic Study of Cystic Fibrosis. Regelmann WE, et al. Among authors: pasta dj. Pediatr Pulmonol. 2013 Jul;48(7):649-57. doi: 10.1002/ppul.22658. Epub 2012 Sep 4. Pediatr Pulmonol. 2013. PMID: 22949088 Free PMC article.
The presence of these four characteristic signs and symptoms is useful to define pulmonary exacerbations in young children with CF that respond to antibiotic treatment in the short-term and influence long-term prognosis....
The presence of these four characteristic signs and symptoms is useful to define pulmonary exacerbations in young children with CF that resp …
Oral, inhaled, and intravenous antibiotic choice for treating pulmonary exacerbations in cystic fibrosis.
Wagener JS, Rasouliyan L, VanDevanter DR, Pasta DJ, Regelmann WE, Morgan WJ, Konstan MW; Investigators and Coordinators of the Epidemiologic Study of Cystic Fibrosis. Wagener JS, et al. Among authors: pasta dj. Pediatr Pulmonol. 2013 Jul;48(7):666-73. doi: 10.1002/ppul.22652. Epub 2012 Aug 8. Pediatr Pulmonol. 2013. PMID: 22888106 Free PMC article.
METHODS: We used 2003-2005 data from the Epidemiologic Study of Cystic Fibrosis to examine antibiotic treatment and the immediate and long-term lung function change associated with clinician reported PExs. ...
METHODS: We used 2003-2005 data from the Epidemiologic Study of Cystic Fibrosis to examine antibiotic treatment and the immediate and long- …
Key findings and clinical implications from The Epidemiology and Natural History of Asthma: Outcomes and Treatment Regimens (TENOR) study.
Chipps BE, Zeiger RS, Borish L, Wenzel SE, Yegin A, Hayden ML, Miller DP, Bleecker ER, Simons FE, Szefler SJ, Weiss ST, Haselkorn T; TENOR Study Group. Chipps BE, et al. J Allergy Clin Immunol. 2012 Aug;130(2):332-42.e10. doi: 10.1016/j.jaci.2012.04.014. Epub 2012 Jun 12. J Allergy Clin Immunol. 2012. PMID: 22694932 Free PMC article.
Regardless of age, patients with severe or difficult-to-treat asthma demonstrated high rates of health care use and substantial asthma burden despite receiving multiple long-term controller medications. Recent exacerbation history was the strongest predictor of future asth …
Regardless of age, patients with severe or difficult-to-treat asthma demonstrated high rates of health care use and substantial asthma burde …
17 results