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Background: Chronic rhinosinusitis (CRS) and olfactory dysfunction (OD) are prevalent disease complications in people with cystic fibrosis. These understudied comorbidities significantly impact quality of life. The impact of highly effective modulator therapy (HEMT) in young children with cystic fibrosis (YCwCF) on these disease complications is unknown.

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Article Synopsis
  • Cystic fibrosis (CF) is often complicated by chronic rhinosinusitis (CRS), and while medical treatments exist, they may not always be effective for some patients, leading to the need for endoscopic sinus surgery.
  • This study aims to compare the outcomes of endoscopic sinus surgery versus continued medical treatment for individuals with CF and CRS, assessing various factors such as pulmonary function, quality of life, and depression.
  • The research is underway across nine CF centers in the U.S., and findings will contribute to guidelines for treating CF patients with CRS.
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Background: Sociodemographic status (SDS) including race/ethnicity and socioeconomic status as approximated by education, income, and insurance status impact pulmonary disease in people with cystic fibrosis (PwCF). The relationship between SDS and chronic rhinosinusitis (CRS) remains understudied.

Methods: In a prospective, multi-institutional study, adult PwCF completed the 22-Question SinoNasal Outcome Test (SNOT-22), Smell Identification Test (SIT), Questionnaire of Olfactory Disorder Negative Statements (QOD-NS), and Cystic Fibrosis Questionnaire-Revised (CFQ-R).

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Article Synopsis
  • The study aimed to identify factors that predict improvement in chronic rhinosinusitis symptoms, measured by the SNOT-22, after initiating highly effective modulator therapy in adults with cystic fibrosis.
  • Data from 184 participants showed that worse initial SNOT-22 scores, having the F508del mutation, and not having prior modulator therapy were linked to greater symptom improvement post-therapy.
  • The minimal clinically important difference (MCID) for the SNOT-22 in this population was determined to be 8.5 points, indicating significant symptom improvement, and the test demonstrated strong internal consistency.
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Background: Many people with cystic fibrosis (PwCF) have chronic rhinosinusitis (CRS). CRS requires additional management beyond that of pulmonary disease and leads to increased utilization of healthcare resources. Elexacaftor/tezacaftor/ivacaftor (ETI) is a highly effective modulator therapy that has been shown to improve CRS in PwCF.

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