ivacaftor

a CFTR potentiator; structure in first source

Also Known As:

3-quinolinecarboxamide, N-(2,4-bis(1,1-dimethylethyl)-5-hydroxyphenyl)-1,4-dihydro-4-oxo-; Kalydeco; N-(2,4-bis(1,1-dimethylethyl)-5-hydroxyphenyl)-4-oxo-1,4-dihydroquinoline-3-carboxamide; N-(2,4-di-tert-butyl-5-hydroxyphenyl)-4-oxo-1,4-dihydroquinoline-3-carboxamide; VX-770

Networked: 459 relevant articles (73 outcomes, 95 trials/studies)

Relationship Network

Bio-Agent Context: Research Results

Experts

1.	Rowe, Steven M: 34 articles (11/2022 - 11/2010)
2.	McKone, Edward F: 19 articles (01/2022 - 11/2011)
3.	Konstan, Michael W: 16 articles (06/2021 - 11/2010)
4.	Tian, Simon: 15 articles (11/2022 - 01/2017)
5.	Davies, Jane C: 15 articles (07/2022 - 06/2013)
6.	Ratjen, Felix: 14 articles (01/2022 - 11/2011)
7.	Higgins, Mark: 13 articles (11/2022 - 12/2014)
8.	Heltshe, Sonya L: 13 articles (11/2022 - 03/2014)
9.	Wainwright, Claire E: 12 articles (01/2022 - 11/2011)
10.	Waltz, David: 12 articles (01/2022 - 07/2014)

Related Diseases

1.	Cystic Fibrosis (Mucoviscidosis) 09/01/2013 - "In two 48-week, double-blind, phase III trials in patients aged ≥12 (STRIVE) or 6-11 (ENVISION) years with cystic fibrosis and the G551D mutation, oral ivacaftor 150 mg every 12 h significantly improved lung function relative to placebo, when used in combination with standard care. " 01/01/2017 - "Ivacaftor has produced significant improvement in certain individuals with cystic fibrosis (CF), though the full metabolic effects of treatment remain unknown. " 05/01/2023 - "Ivacaftor has shown to be effective in patients with cystic fibrosis (CF) with a G551D mutation. " 01/01/2021 - "The CFTR modulator ivacaftor has been variably effective in treating individuals with cystic fibrosis (CF) who harbor CFTR gating variants such as G551D, as well as other classes of CFTR variants when used with other modulators. " 02/01/2016 - "Ivacaftor has been shown to be a safe, effective treatment for cystic fibrosis in patients aged 6 years or older with a CFTR gating mutation. "
2.	Lung Diseases (Lung Disease) 01/01/2020 - "In patients with CFTR mutations that confer RF with severe lung disease, treatment with Ivacaftor is safe and results in a clinically significant improvement that was evident at 1 month and maintained at 12 months." 07/01/2014 - "Ivacaftor was clinically effective in patients with CF who carry the G551D mutation and have severe pulmonary disease. " 01/01/2019 - "Ivacaftor was safe and effective in patients with CF with severe lung disease and non-G551D gating mutations." 01/01/2021 - "Previous studies have proposed that a variant in SLC26A9 (rs7512462) is associated with lung disease severity and with response to treatment with ivacaftor in individuals with CF who carry G551D or gating variants. " 07/01/2015 - "A clinical trial has suggested that there may be a role for ivacaftor in older patients with R117H-CFTR although this trial did not include patients with very severe CF lung disease [4]. "
3.	Weight Gain 01/01/2023 - "Background: Ivacaftor, the first CFTR modulator drug, leads to significant long-term improvement in lung function and weight gain. " 01/01/2023 - "Conclusion: The weight gain observed in ivacaftor treated pwCF is predominantly secondary to increases in fat mass warranting early counseling of people starting on CFTR-modulating treatment with respect to healthy diet and physical exercise." 01/01/2021 - "Significant weight gain with ivacaftor was noted in children and adults with at least 1 copy of G551D mutation. " 12/01/2016 - "New therapies such as ivacaftor in patients with a G551D mutation allows a weight gain in particular by restoring intestinal pH similar to controls. " 01/01/2016 - "In patients with severe lung disease, ivacaftor was well tolerated and was associated with improved lung function and weight gain."
4.	Inflammation (Inflammations) 12/21/2021 - "Ivacaftor alone improved CFTR activity, and lung function and inflammation within 48 h, and reduced P. " 12/13/2018 - "Ivacaftor, a CFTR potentiator, has improved pulmonary and nutritional status but its effects on the intestinal microbiota and inflammation are unclear. " 06/01/2023 - "Thus, we tested ivacaftor as therapeutic strategy for MI-associated target tissue inflammation that is characterized by CFTR alterations. " 01/01/2022 - "There was no significant change in gut inflammation after partial restoration of CFTR function with ivacaftor, suggesting that excess gut inflammation in CF is multi-factorial in aetiology. " 01/01/2021 - "Therefore, we conclude that administration of ivacaftor resulted in an incomplete resolution of inflammation when rats received an external trigger, suggesting that CFTR activation may not be enough to resolve inflammation in the lungs of patients with CF."
5.	Body Weight (Weight, Body) 10/01/2013 - "In both trials, addition of ivacaftor improved FEV1 by an average of about 10% at week 24 and increased body weight by an average of about 2.5 kg after 1 year. " 01/01/2018 - "Ivacaftor has been shown to improve lung function and body weight in patients with CF and a gating mutation. " 01/01/2016 - "In patients ≤20 years, adjusted mean change from baseline to week 48 in body weight was 4.9 versus 2.2 kg (ivacaftor vs. placebo, p = 0.0008). "

Related Drugs and Biologics

1.	lumacaftor
2.	Cystic Fibrosis Transmembrane Conductance Regulator (Protein, CFTR)
3.	Chlorides
4.	tezacaftor
5.	elexacaftor
6.	Anti-Bacterial Agents (Antibiotics)
7.	ivacaftor drug combination tezacaftor
8.	Colistin (Coly-Mycin)
9.	Methionine (L-Methionine)
10.	Proteins (Proteins, Gene)

Related Therapies and Procedures

1.	Therapeutics
2.	Aftercare (After-Treatment)
3.	Precision Medicine
4.	Enzyme Replacement Therapy
5.	Salvage Therapy