title: Rhinosinusitis in the pediatric patient with cystic fibrosis.
abstract: Cystic fibrosis (CF) is a common autosomal recessive genetic disorder where a deletion mutation and subsequent downstream alteration in transmembrane regulator proteins results in increased mucus viscosity. CF manifests clinically with chronic multisystem inflammation and recurrent infections. Nearly all children with CF have chronic sinusitis, and a large majority will have concurrent sinonasal polyposis. Chronic sinusitis and sinonasal polyposis in pediatric patients with CF can be managed conservatively initially, though most will fail medical management and require surgical intervention. Unfortunately, symptom resolution is marginal and polyp recurrence rates are high. Currently, no cure exists for CF and the mainstay of treatment is to provide symptomatic relief, and minimize disease morbidity.
authors.forename: Christopher
authors.initials: C
authors.lastname: Fundakowski
authors.forename: Rosemary
authors.initials: R
authors.lastname: Ojo
authors.affiliation: 900 NW 17th St., Bascom Palmer Eye Institute, Suite 308, Department of Otolaryngology, University of Miami - Miller School of Medicine, Miami, FL 33136, USA. cfundako@gmail.com.
authors.forename: Ramzi
authors.initials: R
authors.lastname: Younis
mesh_terms: D000900:Anti-Bacterial Agents / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000150:complications* / Q000235:genetics / Q000276:immunology / Q000628:therapy; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics; D004252:DNA Mutational Analysis; D020022:Genetic Predisposition to Disease; D006801:Humans; D058990:Molecular Targeted Therapy / Q000639:trends*; D009154:Mutation; D011552:Pseudomonas Infections / Q000209:etiology* / Q000276:immunology / Q000628:therapy; D011788:Quality of Life; D012008:Recurrence; D012220:Rhinitis / Q000209:etiology* / Q000276:immunology / Q000628:therapy; D012852:Sinusitis / Q000209:etiology* / Q000276:immunology / Q000628:therapy
chemical_list: D000900:Anti-Bacterial Agents; C505032:CFTR protein, human; D019005:Cystic Fibrosis Transmembrane Conductance Regulator