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      Cystic fibrosis chronic rhinosinusitis: A comprehensive review

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          Abstract

          Background:

          Advances in the care of patients with cystic fibrosis (CF) have improved pulmonary outcomes and survival. In addition, rapid developments regarding the underlying genetic and molecular basis of the disease have led to numerous novel targets for treatment. However, clinical and basic scientific research focusing on therapeutic strategies for CF-associated chronic rhinosinusitis (CRS) lags behind the evidence-based approaches currently used for pulmonary disease.

          Methods:

          This review evaluates the available literature and provides an update concerning the pathophysiology, current treatment approaches, and future pharmaceutical tactics in the management of CRS in patients with CF.

          Results:

          Optimal medical and surgical strategies for CF CRS are lacking because of a dearth of well-performed clinical trials. Medical and surgical interventions are supported primarily by level 2 or 3 evidence and are aimed at improving clearance of mucus, infection, and inflammation. A number of novel therapeutics that target the basic defect in the cystic fibrosis transmembrane conductance regulator channel are currently under investigation. Ivacaftor, a corrector of the G551D mutation, was recently approved by the Food and Drug Administration. However, sinonasal outcomes using this and other novel drugs are pending.

          Conclusion:

          CRS is a lifelong disease in CF patients that can lead to substantial morbidity and decreased quality of life. A multidisciplinary approach will be necessary to develop consistent and evidence-based treatment paradigms.

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          Most cited references120

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          EPOS 2012: European position paper on rhinosinusitis and nasal polyps 2012. A summary for otorhinolaryngologists

          The European Position Paper on Rhinosinusitis and Nasal Polyps 2012 is the update of similar evidence based position papers published in 2005 and 2007. The document contains chapters on definitions and classification, we now also proposed definitions for difficult to treat rhinosinusitis, control of disease and better definitions for rhinosinusitis in children. More emphasis is placed on the diagnosis and treatment of acute rhinosinusitis. Throughout the document the terms chronic rhinosinusitis without nasal polyps (CRSsNP) and chronic rhinosinusitis with nasal polyps (CRSwNP) are used to further point out differences in pathophysiology and treatment of these two entities. There are extensive chapters on epidemiology and predisposing factors, inflammatory mechanisms, (differential) diagnosis of facial pain, genetics, cystic fibrosis, aspirin exacerbated respiratory disease, immunodeficiencies, allergic fungal rhinosinusitis and the relationship between upper and lower airways. The chapters on paediatric acute and chronic rhinosinusitis are totally rewritten. Last but not least all available evidence for management of acute rhinosinusitis and chronic rhinosinusitis with or without nasal polyps in adults and children is analyzed and presented and management schemes based on the evidence are proposed. This executive summary for otorhinolaryngologists focuses on the most important changes and issues for otorhinolaryngologists. The full document can be downloaded for free on the website of this journal: http://www.rhinologyjournal.com.
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            Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA.

            Overlapping complementary DNA clones were isolated from epithelial cell libraries with a genomic DNA segment containing a portion of the putative cystic fibrosis (CF) locus, which is on chromosome 7. Transcripts, approximately 6500 nucleotides in size, were detectable in the tissues affected in patients with CF. The predicted protein consists of two similar motifs, each with (i) a domain having properties consistent with membrane association and (ii) a domain believed to be involved in ATP (adenosine triphosphate) binding. A deletion of three base pairs that results in the omission of a phenylalanine residue at the center of the first predicted nucleotide-binding domain was detected in CF patients.
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              Differentiation of chronic sinus diseases by measurement of inflammatory mediators.

              Chronic rhinosinusitis (CRS) clinically is a heterogeneous group of sinus diseases, which may cover different disease entities, or may represent a disease continuum. Studying inflammatory cells and mediators in clearly defined disease subgroups may lead to a better differentiation of chronic sinus diseases. Sinonasal mucosal tissue from 10 nasal polyp (NP) patients, 13 cystic fibrosis patients (CF-NP), eight CRS subjects without polyps, and nine control patients were stained for CD3, CD25, CD68, CD20, myeloperoxidase (MPO), CD138 and tissue homogenates were assayed for eotaxin, interleukin (IL)-1beta, IL-2sRalpha, IL-5, interferon (IFN)-gamma, IL-8, transforming growth factor (TGF)-beta1, tumor necrosis factor-alpha, and MPO by enzyme-linked immunosorbent assay or UNICAP system. Nasal polyp and CF-NP showed increased numbers and activation of T cells, while only NP displayed an increase in plasma cells. Nasal polyp had significantly higher levels of eosinophilic markers [eosinophils, eotaxin, and eosinophil cationic protein (ECP)] compared with CRS, controls and CF-NP. Chronic rhinosinusitis was characterized by a Th1 polarization with high levels of IFN-gamma and TGF-beta, while NP showed a Th2 polarization with high IL-5 and immunoglobulin (Ig) E concentrations. Nasal polyp and CF-NP were discriminated by edema from CRS and controls, with CF-NP displaying a very prominent neutrophilic inflammation. Based on cellular and mediator profiles, we suggest that CRS, NP, and CF-NP are distinct disease entities within the group of chronic sinus diseases.
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                Author and article information

                Journal
                Am J Rhinol Allergy
                Am J Rhinol Allergy
                rhinol
                American Journal of Rhinology & Allergy
                OceanSide Publications, Inc. (Providence, RIUSA )
                1945-8924
                1945-8932
                Sep-Oct 2013
                : 27
                : 5
                : 387-395
                Affiliations
                [1]From the Departments of 1Surgery/Division of Otolaryngology and
                [2] 2Medicine, and
                [3]the 3Gregory Fleming James Cystic Fibrosis Research Center, University of Alabama at Birmingham, Birmingham, Alabama
                Author notes
                Address correspondence and reprint requests to Bradford A. Woodworth, M.D., BDB 563, 1530 3rd Avenue S, Birmingham, AL 35294-0012 E-mail address: bwoodwo@ 123456hotmail.com
                Article
                AJRA117-12
                10.2500/ajra.2013.27.3919
                3899543
                24119602
                f30a4ef4-ce38-4b52-8695-589108e9d636
                Copyright © 2013, OceanSide Publications, Inc., U.S.A.

                This publication is provided under the terms of the Creative Commons Public License ("CCPL" or "License"), in attribution 3.0 unported, further described at http://creativecommons.org/license/by/3.0/legalcode. The work is protected by copyright and/or other applicable law. Any use of the work other then as authorized under this license or copyright law is prohibited

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                Immunology
                ataluren,cftr,chloride secretion,chronic sinusitis,cystic fibrosis,dornase alpha,endoscopic medial maxillectomy,functional endoscopic sinus surgery,ivacaftor,lumacaftor,macrolide,mucociliary clearance,pseudomonas,ptc124,rhinosinusitis,saline irrigation,sinus surgery,transepithelial ion transport,vx-770,vx-809

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