Sinonasal Manifestations in Cystic Fibrosis
نویسندگان
چکیده
Cystic fibrosis is a genetic disease, characterized by accumulation of thickened mucous secretions in exocrine glands. Although the major clinical manifestations of the disease are pancreatic and pulmonary disease, the majority of cystic fibrosis patients will develop sinonasal manifestations as well. This paper outlines the etiology, evaluation, and management of the nasal and sinus manifestations in patients with cystic fibrosis.
منابع مشابه
[Relation between rhinosinusitis and bronchiectasis].
The nose and lungs have both histological and functional similarities and differences. Sinonasal and bronchial involvement are associated in many diseases. Cystic fibrosis, primary ciliary dyskinesia, Young's syndrome, and alpha-1 antitrypsin deficiency are diseases in which bronchiectasis and rhinosinusitis are both present. This review considers the diseases in which bronchiectasis occurs alo...
متن کاملUpper airway 2: Bronchiectasis, cystic fibrosis and sinusitis.
The nose and paranasal sinuses are contiguous with the lower respiratory tract. Patients with bronchiectasis and cystic fibrosis commonly have sinonasal disease, which is thought to have the same aetiology and pathophysiology as the chronic lung disease. Despite this, the conditions are rarely considered together and there is very little literature on the treatment of sinonasal disease in bronc...
متن کاملIvacaftor and sinonasal pathology in a cystic fibrosis patient with genotype delta F508/S1215N
In patients with Cystic Fibrosis and a type III mutation, ivacaftor (Kalydeco(®), Vertex) can increase the opening time of the CFTR channel and improve chloride transport. Research showed significant improvement of lung function and increase in weight following ivacaftor use. However, ivacaftor showed to have adverse events on the sinonasal system as well, such as upper respiratory tract infect...
متن کاملAnalysis of CFTR Gene Mutations in Children with Cystic Fibrosis, First Report from North-East of Iran
Objective(s): More than 1500 registered mutations in cystic fibrosis transmembrane regulator (CFTR) gene are responsible for dysfunction of an ion channel protein and a wide spectrum of clinical manifestations in patients with cystic fibrosis (CF). This study was performed to investigate the frequency of a number of well-known CFTR mutations in North Eastern Iranian CF patients. Material and...
متن کاملChildren with cystic fibrosis: who should visit the otorhinolaryngologist?
BACKGROUND Sinonasal complications are common in children with cystic fibrosis (CF). Generally, those children with persistent symptoms of sinonasal polyposis are referred to an otorhinolaryngologic (ORL) physician for sinus surgery. Several studies have reported differences in clinical characteristics between CF patients with and without sinonasal polyps. OBJECTIVES To predict the presence o...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
دوره 2012 شماره
صفحات -
تاریخ انتشار 2012