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Cystic fibrosis sinus disease

Paolo Luparello, Maria S Lazio, Luca Voltolini, Beatrice Borchi, Giovanni Taccetti, Giandomenico Maggiore
PURPOSE: Cystic Fibrosis (CF) is the most common autosomal recessive disease in Caucasian population. Due to its pathological mechanism, chronic rhino sinusitis (CRS) associated or not with nasal polyposis usually occurs in adults and affects close to one-half of all CF patients. The goal of our work was to evaluate the impact of Endoscopic Sinus Surgery (ESS) in the quality of life (QoL) of the CF patients and demonstrate an improvement of the functional outcomes in the patients underwent the surgical procedure rather than in the not treated ones, particulary in lung transplant patients...
January 28, 2019: European Archives of Oto-rhino-laryngology
Nauman Chaudary
Cystic fibrosis (CF) is an autosomal recessive genetic disease characterized by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR). CFTR is a chloride channel responsible for ion flow across epithelial surfaces of lung, sinuses, pancreas, intestine, and liver. Researchers have grouped CFTR genetic mutations into various protein defects: reduced protein synthesis (class 1 mutations), abnormal protein folding and maturation (class 2 mutation), and abnormal gating (class 3 mutation). These mutations usually present as severe forms of CF due to complete absence of CFTR at cell surfaces...
2018: Therapeutics and Clinical Risk Management
Ashleigh A Halderman, Stella Lee, Nyall R London, Andrew Day, Raksha Jain, John A Moore, Sandra Y Lin
OBJECTIVE: Understanding of how specific mutations impact the cystic fibrosis transmembrane conductance regulator (CFTR) protein has given rise to the classification of CF patients into low-risk and high-risk genotypes. Few prior studies have investigated differences in sinonasal disease between low-risk and high-risk CF genotypes. This multi-institutional review aimed to evaluate radiographic sinus disease severity based on genotype. METHODS: A review was conducted on adult patients with CF evaluated between 2005 to 2017 at three academic institutions...
December 14, 2018: Laryngoscope
Ryan G Short, Tina D Tailor
History A 31-year-old woman with a history of bilateral orthotopic lung transplantation performed 10 months earlier for cystic fibrosis presented for a routine follow-up appointment, with her chief symptom being a cough. The cough started approximately 1 month prior to this appointment and was minimally productive of clear to yellow phlegm. In addition to her cough, she reported increased sinus congestion and a sensation of "something in her upper chest." She denied shortness of breath, wheezing, hemoptysis, or cigarette smoking...
December 2018: Radiology
Brittany N Burton, Sapideh Gilani, Milli Desai, Robert Saddawi-Konefka, Lindia Willies-Jacobo, Rodney A Gabriel
OBJECTIVES: Pediatric sinus surgery is indicated for a wide range of sinonasal and skull base pathologies, but it is most commonly performed for recalcitrant chronic rhinosinusitis or complicated acute sinusitis. The authors aim to report medical risk factors of morbidity and mortality following inpatient sinus surgery in the pediatric population. METHODS: Using data from the Kids' Inpatient Database from 2003 to 2012, patients with International Classification of Diseases, Ninth Revision, procedure codes for primary sinus surgery were identified...
October 17, 2018: Annals of Otology, Rhinology, and Laryngology
L Regard, H Lafoeste, C Martin, G Chassagnon, P-R Burgel
Cystic fibrosis (CF) is a genetic disease with pulmonary involvement being predominant and often leading to respiratory failure and premature death. Non-pulmonary complications related to CF transmembrane conductance regulator (CFTR) defect are numerous and account for significant morbidity (exocrine pancreatic insufficiency, diabetes, CF-related liver disease, chronic sinusitis, osteoporosis). Improvement in patients' care led to a continuous increase in life expectancy, with a subsequent increase in the number of adult CF patients worldwide...
October 2018: Revue de Pneumologie Clinique
Jean-Nicolas Gallant, Margaret B Mitchell, Frank W Virgin
PURPOSE OF REVIEW: There is a lack of consensus with regards to the diagnosis and treatment of sinus disease in children with cystic fibrosis. Here, we review literature from the past 18 months in order to highlight the way forward in this contentious field. RECENT FINDINGS: Most of the literature (from the past 18 months) on sinus disease in pediatric cystic fibrosis focused on treatment approaches, bacteriology and immunology, and health-related quality-of-life (HRQOL) instruments...
December 2018: Current Opinion in Otolaryngology & Head and Neck Surgery
Steven D Pletcher, Andrew N Goldberg, Emily K Cope
OBJECTIVES/HYPOTHESIS: To determine the relationship between mucosal-associated sinus and bronchial microbiota in cystic fibrosis (CF) patients compared to non-CF patients with chronic rhinosinusitis (CRS). STUDY DESIGN: Case series. METHODS: We examined the microbial composition of 52 paired sinus and bronchial brushings from 26 patients with CRS. Paired airway samples from nine subjects with CF were compared with samples from 17 non-CF-CRS disease control patients...
October 3, 2018: Laryngoscope
Do-Yeon Cho, Shaoyan Zhang, Ahmed Lazrak, Jessica W Grayson, Jaime A Peña Garcia, Daniel F Skinner, Dong Jin Lim, Calvin Mackey, Catherine Banks, Sadis Matalon, Bradford A Woodworth
BACKGROUND: Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene result in defective Cl- transport and cause chronic bacterial infections in the upper and lower airways of cystic fibrosis (CF) patients. Ivacaftor is a CFTR potentiator that improves Cl- transport in CF patients with at least 1 copy of the G551D mutation. Resveratrol is also a potent CFTR potentiator that increases determinants of mucociliary transport. The objective of this study is to determine whether resveratrol and ivacaftor improve Cl- secretion in G551D CFTR over either agent alone...
August 27, 2018: International Forum of Allergy & Rhinology
M Kérimian, P-L Bastier, N Réville, S Fierens, L de Gabory
OBJECTIVES: To assess the feasibility of bilateral radical ethmoidectomy in ambulatory surgery by risk analysis, and to calculate possible medico-economic savings. METHODS: This study was performed retrospectively over a 2-year period and prospectively for 1 year. It included all patients undergoing bilateral ethmoidectomy, associated to sphenoidotomy and/or septoplasty or not, in a university hospital department. Data were collected on demographics, disease etiology, previous surgery, operative details, postoperative course, complications and satisfaction assessed by questionnaire at days 1 and 30...
August 17, 2018: European Annals of Otorhinolaryngology, Head and Neck Diseases
Jesada Kanjanaumporn, Peter H Hwang
Background The concept of unified airway disease has linked bronchiectasis with chronic rhinosinusitis (CRS), much in the same way as in asthma and CRS. Although the outcomes of endoscopic sinus surgery (ESS) on comorbid asthma have been relatively well studied, the outcomes of ESS on comorbid bronchiectasis have rarely been examined. Objective We sought to determine sinonasal and pulmonary clinical outcomes of ESS in bronchiectasis patients with CRS. Method We reviewed all bronchiectasis patients who had ESS for CRS at our institution from 2006 to present...
September 2018: American Journal of Rhinology & Allergy
Letizia Corinna Morlacchi, Mark Greer, Igor Tudorache, Francesco Blasi, Tobias Welte, Axel Haverich, Jochen G Mainz, Jens Gottlieb
INTRODUCTION: Sinus disease (SD) in cystic fibrosis (CF) is a known risk factor for disease progression, the upper airways (UAW) being a site of primary colonization with Pseudomonas aeruginosa. UAW may function as reservoir for graft colonization after lung transplantation (LuTx), increasing risk of rejection. Aims of this study were to assess the burden of sinus disease in CF LuTx recipients, considering patient-reported symptoms, endoscopically documented signs and microbiological isolates, comparing colonization between upper and lower airways...
October 2018: Transplant Infectious Disease: An Official Journal of the Transplantation Society
S Shen, C S Wang
Macrolides are a type of antibiotics with macrocyclic lactone ring, which have been commonly used in the treatment of diffuse panbronchiolitis, chronic obstructive pulmonary disease, bronchial asthma, cystic fibrosis and other diseases. Macrolides not only have effect on antibacterial, but also effect on anti-inflammatory and immunoregulation. Chronic rhinosinusitis (CRS) is a common disease entity of infectious and inflammatory diseases that involved in nasal cavity and nasal sinuses, with various clinical phenotypes...
May 2018: Journal of Clinical Otorhinolaryngology, Head, and Neck Surgery
Whittney A Warren, Domingo Franco-Palacios, Christopher S King, Oksana A Shlobin, Steven D Nathan, Shalika B Katugaha, Haresh Mani, A Whitney Brown
A 24-year-old woman with ΔF508/Y1092X cystic fibrosis (CF) complicated by severe obstructive lung disease (FEV1 of 30% predicted) was admitted for IV antibiotics for planned sinus surgery resulting from severe chronic sinusitis causing frequent exacerbations and declining lung function. She had persistent airway infection with multidrug-resistant Pseudomonas aeruginosa, methicillin-resistant Staphylococcus aureus, and growth of a fungus presumed to be an airway colonizer, identified as Stephanoascus ciferrii 1 year before presentation...
March 2018: Chest
Olga L Voronina, Marina S Kunda, Natalia N Ryzhova, Ekaterina I Aksenova, Natalia E Sharapova, Andrey N Semenov, Elena L Amelina, Alexandr G Chuchalin, Alexandr L Gintsburg
BACKGROUND: Microbes infecting cystic fibrosis patients' respiratory tract are important in determining patients' functional status. Representatives of Burkholderiales order are the most dangerous. The goal of our investigation was to reveal the diversity of Burkholderiales, define of their proportion in the microbiome of various parts of respiratory tract and determine the pathogenicity of the main representatives. RESULTS: In more than 500 cystic fibrosis patients, representing all Federal Regions of Russia, 34...
February 9, 2018: BMC Genomics
Michal Shteinberg, Najwan Nassrallah, Jenny Jrbashyan, Nechama Uri, Nili Stein, Yochai Adir
The association of bronchiectasis with chronic rhinosinusitis (CRS) has been reported. However, apart from primary ciliary dyskinesia (PCD) and cystic fibrosis (CF), predisposing conditions have not been established. We aimed to define clinical and laboratory features that differentiate patients with bronchiectasis with upper airway symptoms (UASs) and without PCD from patients without UASs. We reviewed charts of adults with bronchiectasis, excluding CF and PCD. UASs were defined as nasal discharge most days of the year, sinusitis or nasal polyps...
January 2018: ERJ Open Research
Kiranya E Tipirneni, Shaoyan Zhang, Do-Yeon Cho, Jessica Grayson, Daniel F Skinner, Calvin Mackey, Lindsay Moore, Denzel Cole, Catherine G Banks, Bradford A Woodworth
BACKGROUND: Chronic rhinosinusitis (CRS) may be initiated by innately impaired host defense mechanisms that predispose the upper airways to infection. Recent evidence suggests tethering of submucosal gland mucus strands represents an inciting event within cystic fibrosis (CF) airways, occurring prior to onset of chronic infection. Submucosal gland hypertrophy and defective mucociliary clearance (MCC) are present in actively inflamed sinuses, but mucus strand velocity may also be affected as a secondary event, further contributing to chronic disease...
April 2018: International Forum of Allergy & Rhinology
Kathryn L Kreicher, Michael J Bauschard, Clarice S Clemmens, Concetta Maria Riva, Ted A Meyer
BACKGROUND: The purpose of this study was to evaluate hearing impairment in pediatric patients with cystic fibrosis (CF). METHODS: This is a retrospective analysis of the AudGen database generated by Children's Hospital of Philadelphia. Audiograms were analyzed for type of hearing loss (HL), pure-tone-average (PTA), laterality, and change in hearing over time. Medical charts were reviewed to identify factors that influence development and progression of hearing loss...
May 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
Kevin J Choi, Tracy Z Cheng, Adam L Honeybrook, Alice L Gray, Laurie D Snyder, Scott M Palmer, Ralph Abi Hachem, David W Jang
BACKGROUND: Lung transplantation has revolutionized the treatment of end-stage pulmonary disease due to cystic fibrosis. However, infection of the transplanted lungs can lead to serious complications, including graft failure and death. Although many of these patients have concurrent sinusitis, it is unclear whether bacteria from the sinuses can infect the allograft. METHODS: This is a single-institution retrospective study of all patients who underwent lung transplantation for cystic fibrosis from 2005 to 2015 at Duke University Hospital...
March 2018: International Forum of Allergy & Rhinology
Jacob Nelson, Peter Karempelis, Jordan Dunitz, Ryan Hunter, Holly Boyer
BACKGROUND: Indirect evidence suggests that sinonasal secretions are aspirated into the lungs of patients with cystic fibrosis (CF), contributing to infection, subsequent tissue damage, and decreased lung function. Our objective is to determine whether sinonasal secretions are transferred to the lungs in patients with CF-related sinus disease and healthy subjects, particularly in the recumbent position and during sleep. METHODS: We performed a prospective, controlled trial to detect pulmonary aspiration of radiolabeled albumin applied to the nasal mucosa of study subjects with chronic sinusitis related to CF and control subjects without sinus disease...
March 2018: International Forum of Allergy & Rhinology
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