scholarly journals Effect of highly effective modulator treatment on sinonasal symptoms in cystic fibrosis

2020 ◽  
Author(s):  
Emily DiMango ◽  
Jonathan Overdevest ◽  
Claire Keating ◽  
Sarah Fracasso Francis ◽  
David Dansky ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Highly Effective ◽  
Sinonasal Symptoms

scholarly journals Management of Individual Patient Expectations When Starting with Highly Effective CFTR Modulators

2021 ◽  
Vol 11 (8) ◽  
pp. 811
Author(s):  
Bente L. Aalbers ◽  
Inez Bronsveld ◽  
Regina W. Hofland ◽  
Harry G. M. Heijerman
Keyword(s):  
Quality Of Life ◽  
Cystic Fibrosis ◽  
Lung Damage ◽  
Cell Models ◽  
Highly Effective ◽  
Near Future ◽  
Cftr Modulators ◽  
Sinonasal Symptoms ◽  
Severe Lung

Highly effective CFTR modulators such as elexacaftor/tezacaftor/ivacaftor (ELE/TEZ/IVA will become available for an increasing number of people with cystic fibrosis (pwCF) in the near future. Before the start of this therapy, many questions may arise concerning the expected effects. We assembled the currently available data from the literature about ELE/TEZ/IVA that focused on commonly asked questions from patients. Overall, the literature so far presents a very hopeful prospect of effects, not only on lung function, but also on nutritional status, sinonasal symptoms and quality of life. The effects in patients with pwCF with severe lung damage are also favorable. Treatment is generally well tolerated. In some cases, patient-derived cell models can help in predicting the effects for individual patients.

Importance to question sinonasal symptoms and to perform rhinoscopy and rhinomanometry in cystic fibrosis patients

2016 ◽  
Vol 52 (2) ◽  
pp. 167-174 ◽  
Author(s):  
J.M. Bock ◽  
M. Schien ◽  
C. Fischer ◽  
L. Naehrlich ◽  
M. Kaeding ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Sinonasal Symptoms

Effect of highly effective modulator therapy on quality of life in adults with cystic fibrosis

2020 ◽  
Author(s):  
Emily DiMango ◽  
Daniel B. Spielman ◽  
Jonathan Overdevest ◽  
Claire Keating ◽  
Sarah Fracasso Francis ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Cystic Fibrosis ◽  
Highly Effective

Cystic Fibrosis and the Sinonasal Tract

1996 ◽  
Vol 105 (4) ◽  
pp. 329-330 ◽  
Author(s):  
John G. Batsakis ◽  
Adel K. El-Naggar
Keyword(s):  
Cystic Fibrosis ◽  
Surgical Treatment ◽  
Respiratory Tract ◽  
Paranasal Sinuses ◽  
Lower Respiratory Tract ◽  
Sinonasal Tract ◽  
Inherited Diseases ◽  
Radiologic Examination ◽  
Sinonasal Symptoms ◽  
All Treatment

Cystic fibrosis is considered to be one of the most common fatal inherited diseases. Sinonasal manifestations are not outstanding as compared with those in the lower respiratory tract. Panopacification of the paranasal sinuses is, however, almost universal on radiologic examination of patients, although many of these patients do not have sinonasal symptoms. Approximately 10% to 20% of cystic fibrosis patients will eventually require surgical treatment of their sinuses. All treatment, including surgery, is transiently effective; none is curative.

scholarly journals 94: Assessing symptom burden and quality of life in patients with cystic fibrosis in the era of highly effective modulator therapy

2021 ◽  
Vol 20 ◽  
pp. S47-S48
Author(s):  
A. Perez ◽  
K. Bischoff ◽  
D. Zhang ◽  
D. Dawson ◽  
M. Israni ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Cystic Fibrosis ◽  
Symptom Burden ◽  
Highly Effective

scholarly journals Changes of Proteases, Antiproteases, and Pathogens in Cystic Fibrosis Patients’ Upper and Lower Airways after IV-Antibiotic Therapy

2015 ◽  
Vol 2015 ◽  
pp. 1-16 ◽  
Author(s):  
Ulrike Müller ◽  
Julia Hentschel ◽  
Wibke K. Janhsen ◽  
Kerstin Hünniger ◽  
Uta-Christina Hipler ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Antibiotic Therapy ◽  
Proteolytic Enzymes ◽  
Before And After ◽  
Lower Airways ◽  
Time Changes ◽  
First Time ◽  
Antibiotic Courses ◽  
Sinonasal Symptoms ◽  
Multiplex Bead

Background. In cystic fibrosis (CF) the upper (UAW) and lower airways (LAW) are reservoirs for pathogens likePseudomonas aeruginosa. The consecutive hosts’ release of proteolytic enzymes contributes to inflammation and progressive pulmonary destruction. Objectives were to assess dynamics of protease : antiprotease ratios and pathogens in CF-UAW and LAW sampled by nasal lavage (NL) and sputum before and after intravenous- (IV-) antibiotic therapy.Methods. From 19 IV-antibiotic courses of 17 CF patients NL (10 mL/nostril) and sputum were collected before and after treatment. Microbiological colonization and concentrations of NE/SLPI/CTSS (ELISA) and MMP-9/TIMP-1 (multiplex bead array) were determined. Additionally, changes of sinonasal symptoms were assessed (SNOT-20).Results. IV-antibiotic treatment had more pronounced effects on inflammatory markers in LAW, whereas trends to decrease were also found in UAW. Ratios of MMP-9/TIMP-1 were higher in sputum, and ratios of NE/SLPI were higher in NL. Remarkably, NE/SLPI ratio was 10-fold higher in NL compared to healthy controls. SNOT-20 scores decreased significantly during therapy(P=0.001).Conclusion. For the first time, changes in microbiological patterns in UAW and LAW after IV-antibiotic treatments were assessed, together with changes of protease/antiprotease imbalances. Delayed responses of proteases and antiproteases to IV-antibiotic therapy were found in UAW compared to LAW.

Current evidence on the effect of highly effective CFTR modulation on interleukin-8 in cystic fibrosis

2021 ◽  
Author(s):  
Michael Williamson ◽  
Michelle Casey ◽  
Claudie Gabillard-Lefort ◽  
Aram Alharbi ◽  
Yu Qing Jolene Teo ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Interleukin 8 ◽  
Current Evidence ◽  
Highly Effective
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