scholarly journals Chronic rhinosinusitis in cystic fibrosis: a review of therapeutic options

2021 ◽  
Author(s):  
Joanna Krajewska ◽  
Krzysztof Zub ◽  
Adam Słowikowski ◽  
Tomasz Zatoński
Keyword(s):  
Cystic Fibrosis ◽  
Chronic Rhinosinusitis ◽  
Current Knowledge ◽  
Genetic Defect ◽  
Negative Influence ◽  
Therapeutic Options ◽  
Sinus Surgery ◽  
Surgical Approaches ◽  
Positive Effects ◽  
Nasal Decongestants

Abstract Purpose Chronic rhinosinusitis (CRS) is observed in almost 100% of patients with cystic fibrosis (CF). CF-related CRS treatment is extremely challenging because of the underlying genetic defect leading to its development. CRS in CF is often refractory to standard therapy, while recurrences after surgical treatment are inevitable in the majority of patients. This study provides a precise review of the current knowledge regarding possible therapeutic options for CF-related CRS. Methods The Medline and Web of Science databases were searched without a time limit using the terms “cystic fibrosis” in conjunction with “otorhinolaryngological manifestation”, “rhinology” and “sinusitis”. Results Precise guidelines for CF-induced CRS therapy are lacking due to the lack of large cohort randomized controlled trials. None of the existing therapeutic agents has already been recommended for CRS in CF. Therapy targeting the underlying genetic defect, intranasal dornase alfa administration, and topical delivery of colistin and tobramycin showed promising results in CF-related CRS therapy. Besides the potential effectiveness of nasal steroids, strong recommendations for their usage in CF have not been provided yet. Systemic corticosteroid usage is controversial due to its potential negative influence on pulmonary disease. Ibuprofen revealed some positive effects on CF-related CRS in molecular and small cohort studies. Intranasal irrigation with saline solutions could relieve sinonasal symptoms. Nasal decongestants are not recommended. Endoscopic sinus surgery is the first-line surgical option for refractory CRS. Extensive surgical approaches should be considered as they could improve long-term outcomes in CRS. Conclusion Further studies are warranted to establish consensus for CF-related CRS therapy.

scholarly journals Chronic rhinosinusitis in adult patients with cystic fibrosis: clinical manifestation and therapeutic approaches

2019 ◽  
Vol 29 (3) ◽  
pp. 311-320
Author(s):  
G. L. Shumkova ◽  
E. L. Amelina ◽  
V. M. Svistushkin ◽  
E. V. Sin’kov ◽  
S. A. Krasovskiy ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Surgical Treatment ◽  
Treatment Group ◽  
Chronic Rhinosinusitis ◽  
Paranasal Sinuses ◽  
Endoscopic Sinus Surgery ◽  
Nasal Polyps ◽  
Adult Patients ◽  
Sinus Surgery ◽  
Markers Of Inflammation

The aim of this study was to evaluate prevalence of chronic rhinosinusitis (CRS) and nasal polyps in adult patients with cystic fibrosis (CF) in Russian Federation. Additionally, we investigated the clinical course of CRS and developed the optimal therapeutic strategy.Methods. Three hundred and forty eight CF patients were involved in the study. Physical examination, computed tomography (CT) of paranasal sinuses and audiometry, if needed, were used. CRS and bilateral nasal polyps were diagnosed in 28 patients. Nasal endoscopy, SNOT-20 questionnaire, rhinomanometry, micro - biological examination of sputum and mucus from paranasal sinuses (obtained during puncture or surgery), spirometry, and measurement of serum markers of inflammation were used. Endoscopic sinus surgery was used in 14 patients (the group 1) and others were treated non-surgically (the group 2). Both group were treated during 6 months using intranasal mometasone, mucolytics and antibiotics via PARI SINUSTM nebulizer.Results. An improvement in symptoms, CT signs, rhinomanometry parameters and endoscopic signs was seen in both groups after treatment and was more prominent in the surgical treatment group compared to the non-surgical treatment group. Bacterial load reduction in nasal sinuses, decrease in the rate of pulmonary disease exacerbations, and an improvement in oxygen blood saturation were found in the surgical treatment group only. Treatment of CRS did not affect lung function, sputum microbiology and serum inflammatory markers.Conclusion. Endoscopic sinus surgery followed by intranasal mucolytics and antibacterials is an effective and well-tolerated treatment in adult CF patients with CRS. 

Office-Based Sinus Surgery for Cystic Fibrosis Chronic Rhinosinusitis

ORL ◽  
2020 ◽  
pp. 1-4
Author(s):  
Daniel B. Spielman ◽  
David A. Gudis
Keyword(s):  
Cystic Fibrosis ◽  
Mechanical Ventilation ◽  
Chronic Rhinosinusitis ◽  
Local Anesthesia ◽  
Endoscopic Sinus Surgery ◽  
Care Center ◽  
Academic Medical Center ◽  
Tertiary Care ◽  
Sinus Surgery ◽  
Office Setting

<b><i>Objective:</i></b> Chronic rhinosinusitis (CRS) is nearly ubiquitous in the cystic fibrosis (CF) population, and many patients require multiple endoscopic sinus surgeries throughout their lifetime. Recent studies have demonstrated the profound pulmonary and systemic health benefits of comprehensive CRS treatment. Both endotracheal intubation with mechanical ventilation and inpatient hospital care represent significant risks for CF patients. The goal of this study is to evaluate the safety and feasibility of performing revision endoscopic sinus surgery for CF patients in the outpatient office setting using only local anesthesia to decrease the need for mechanical ventilation and inpatient hospitalization. <b><i>Methods:</i></b> This is a prospective cohort study conducted at a tertiary care academic medical center with a CF Foundation-accredited care center. Patients with CF and refractory CRS despite prior surgery and medical therapy were eligible for inclusion. Comprehensive revision ESS was performed in the office using only local anesthesia. <b><i>Results:</i></b> Five patients were enrolled and underwent revision endoscopic sinus surgery without complication. The average preoperative Sinonasal-Outcome Test-22 score was 52.0 ± 12.1 and the average preoperative Lund-Mackay score was 15.2 ± 3.8. No patients requested aborting the procedure early due to pain, discomfort, or any other reason. No subjects required prolonged observation or postoperative hospital admission. <b><i>Conclusion:</i></b> This prospective pilot study is the first to demonstrate the safety and feasibility of performing comprehensive revision endoscopic sinus surgery for CF patients in the outpatient office setting using only local anesthesia.

scholarly journals Surgical Management of Chronic Rhinosinusitis in Cystic Fibrosis

2019 ◽  
Vol 7 (4) ◽  
pp. 57 ◽  
Author(s):  
Zhong Zheng ◽  
Chetan Safi ◽  
David Gudis
Keyword(s):  
Cystic Fibrosis ◽  
Chronic Rhinosinusitis ◽  
Surgical Management ◽  
Symptom Burden ◽  
Sinus Surgery ◽  
Current Evidence ◽  
Control Disease ◽  
The Impact ◽  
Positive Effect ◽  
Topical Antimicrobial

Cystic fibrosis patients frequently develop chronic rhinosinusitis as a result of their propensity to form inspissated mucus and impairment of mucociliary clearance. They exhibit variable symptom burden even in the setting of positive radiographic and endoscopic findings. Current evidence suggests a positive effect of managing sinonasal disease on pulmonary health. Topical antimicrobial and mucolytic therapies are frequently required to manage the disease with surgery reserved for refractory cases. Endoscopic sinus surgery has been demonstrated to be safe and efficacious in controlling symptoms of chronic rhinosinusitis in patients with comorbid cystic fibrosis. However, the impact of surgery on pulmonary health remains an active area of investigation. In addition, a growing body of research has suggested a more extended surgical approach creating large sinonasal cavities with gravity-dependent drainage pathways, followed by adjuvant medical therapies, as an ideal strategy to optimally control disease and prevent pulmonary exacerbations. In this manuscript, we provide an up-to-date review of current evidence in the surgical management of chronic rhinosinusitis in cystic fibrosis patients.

scholarly journals Stent Hypersensitivity and Infection in Sinus Cavities

2013 ◽  
Vol 4 (3) ◽  
pp. ar.2013.4.0071 ◽  
Author(s):  
Nicholas G. Kounis ◽  
George D. Soufras ◽  
George Hahalis
Keyword(s):  
Chronic Rhinosinusitis ◽  
Granulation Tissue ◽  
Endoscopic Sinus Surgery ◽  
Current Knowledge ◽  
Klebsiella Oxytoca ◽  
Sinus Surgery ◽  
Mucosal Inflammation ◽  
Coagulase Negative Staphylococcus ◽  
Moderate Growth ◽  
Beta Hemolytic Streptococcus

Persistent mucosal inflammation, granulation tissue formation, hypersensitivity, and multifactorial infection are newly described complications of retained drug-eluting stents from endoscopic sinus surgery for refractory rhinosinusitis. In an important report published in Allergy and Rhinology, a 45-year-old male patient suffering from recalcitrant chronic rhinosinusitis underwent functional endoscopic sinus surgery and was found, for the first time, to have steroid-eluting catheters that were inadvertently left in the ethmoid and frontal sinuses. The retained catheters had caused persistent mucosal inflammation and formation of granulation tissue denoting hypersensitivity reaction. These consequences had induced perpetuation of symptoms of chronic rhinosinusitis. Meticulous removal of the retained stents with the nitinol wings from inflamed tissues of the frontal, ethmoidal, and sphenoethmoidal recesses in which they were completely imbedded was successfully performed without polypoid regrowth. Cultures of specimens taken from both left and right stents showed heavy growth of Stenotrophomonas maltophilia and moderate growth of Klebsiella oxytoca, coagulase negative Staphylococcus, and beta-hemolytic Streptococcus anginosus. Fungal infection was not detected. The current knowledge and experience regarding stent hypersensitivity and infection in relation with the use of stents in sinus cavities is reviewed.

scholarly journals Longitudinal analysis of sinus microbiota post endoscopic surgery in patients with cystic fibrosis and chronic rhinosinusitis: a pilot study

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Brett Wagner Mackenzie ◽  
Camila Dassi ◽  
Anitha Vivekanandan ◽  
Melissa Zoing ◽  
Richard G. Douglas ◽  
...  
Keyword(s):  
Gene Expression ◽  
Cystic Fibrosis ◽  
Chronic Rhinosinusitis ◽  
Sampling Period ◽  
Amplicon Sequencing ◽  
Molecular Techniques ◽  
Sinus Surgery ◽  
Lower Airway ◽  
Upper Respiratory Tract ◽  
Microbial Composition

Abstract Background Cystic fibrosis is a debilitating, autosomal recessive disease which results in chronic upper and lower airway infection and inflammation. In this study, four adult patients presenting with cystic fibrosis and chronic rhinosinusitis were recruited. Culture and molecular techniques were employed to evaluate changes in microbial profiles, host gene expression and antimicrobial resistance (AMR) in the upper respiratory tract over time. Methods Swab samples from the sinonasal cavity were collected at the time of surgery and at follow-up clinics at regular time intervals for up to 18 months. Nucleic acids were extracted, and DNA amplicon sequencing was applied to describe bacterial and fungal composition. In parallel, RNA was used to evaluate the expression of 17 AMR genes and two inflammatory markers (interleukins 6 and 8) using custom qPCR array cards. Molecular results were compared with routine sinus and sputum culture reports within each patient. Results Bacterial amplicon sequencing and swab culture reports from the sinonasal cavity were mostly congruent and relatively stable for each patient across time. The predominant species detected in patients P02 and P04 were Pseudomonas aeruginosa, Staphylococcus aureus in patient P03, and a mixture of Enterobacter and S. aureus in patient P01. Fungal profiles were variable and less subject specific than bacterial communities. Increased expressions of interleukins 6 and 8 were observed in all patients throughout the sampling period compared with other measured genes. The most prevalent AMR gene detected was ampC. However, the prevalence of AMR gene expression was low in all patient samples across varying time-points. Conclusions We observed a surprising degree of stability of sinonasal microbial composition, and inflammatory and AMR gene expression across all patients post sinus surgery.

Endonasal Sinus Surgery: Extended versus Limited Approach

2002 ◽  
Vol 16 (4) ◽  
pp. 187-192 ◽  
Author(s):  
Matthias Kuehnemund ◽  
Andrej Lopatin ◽  
Ronald G. Amedee ◽  
Wolf J. Mann
Keyword(s):  
Chronic Rhinosinusitis ◽  
Sinus Surgery ◽  
Surgical Approaches ◽  
Postoperative Results ◽  
Transport Time ◽  
Endonasal Sinus Surgery ◽  
Extended Approach ◽  
Long Term Follow Up

Background This study was designed to investigate whether two different surgical approaches in endonasal sinus surgery lead to different postoperative results in patients with chronic rhinosinusitis. Methods Sixty-five patients suffering from chronic rhinosinusitis were operated on with either a limited approach or an extended approach. They were examined preoperatively and reassessed during 3 months, 6 months, and long-term follow-up visits that included endoscopy, saccharin transport time, and the assessment of symptoms. Results The concept of this study was prospective, randomized, and multicenter (Mainz, Germany, and Moscow, Russia). The results of the two different surgical approaches were compared with each other. Conclusion Surgical results and symptomatology were similar in both groups of patients, suggesting that a conservative approach may be sufficient in most patients.

scholarly journals Role of Protein Kinase A-Mediated Phosphorylation in CFTR Channel Activity Regulation

2021 ◽  
Vol 12 ◽  
Author(s):  
Angela Della Sala ◽  
Giulia Prono ◽  
Emilio Hirsch ◽  
Alessandra Ghigo
Keyword(s):  
Cystic Fibrosis ◽  
Protein Kinase ◽  
Protein Kinase A ◽  
Chloride Transport ◽  
Current Knowledge ◽  
Genetic Defect ◽  
Channel Activity ◽  
The Impact ◽  
Kinase A ◽  
R Domain

Cystic fibrosis transmembrane conductance regulator (CFTR) is an anion channel expressed on the apical membrane of epithelial cells, where it plays a pivotal role in chloride transport and overall tissue homeostasis. CFTR constitutes a unique member of the ATP-binding cassette transporter superfamily, due to its distinctive cytosolic regulatory (R) domain carrying multiple phosphorylation sites that allow the tight regulation of channel activity and gating. Mutations in the CFTR gene cause cystic fibrosis, the most common lethal autosomal genetic disease in the Caucasian population. In recent years, major efforts have led to the development of CFTR modulators, small molecules targeting the underlying genetic defect of CF and ultimately rescuing the function of the mutant channel. Recent evidence has highlighted that this class of drugs could also impact on the phosphorylation of the R domain of the channel by protein kinase A (PKA), a key regulatory mechanism that is altered in various CFTR mutants. Therefore, the aim of this review is to summarize the current knowledge on the regulation of the CFTR by PKA-mediated phosphorylation and to provide insights into the different factors that modulate this essential CFTR modification. Finally, the discussion will focus on the impact of CF mutations on PKA-mediated CFTR regulation, as well as on how small molecule CFTR regulators and PKA interact to rescue dysfunctional channels.

Sign in / Sign up

Export Citation Format

Share Document