Literature DB >> 24210900

Sinonasal manifestations of cystic fibrosis: a correlation between genotype and phenotype?

M C Berkhout1, C J van Rooden2, E Rijntjes3, W J Fokkens4, L H el Bouazzaoui5, H G M Heijerman5.   

Abstract

BACKGROUND: Patients with Cystic Fibrosis are prone to develop sinonasal disease. Studies in genotype-phenotype correlations for sinonasal disease are scarce and inconclusive.
METHODS: In this observational study several aspects of sinonasal disease were investigated in 104 adult patients with CF. In each patient a disease specific quality of life questionnaire (RSOM-31), nasal endoscopy and a CT scan of the paranasal sinuses were performed. Patients were divided into two groups, class I-III mutations and class IV-V mutations, based on their CFTR mutations.
RESULTS: The prevalence of rhinosinusitis in adult patients with CF was 63% and the prevalence of nasal polyps 25%. Patients with class I-III mutations had significantly smaller frontal sinuses, sphenoid sinuses, more opacification in the sinonasal area and more often osteitis/neoosteogenesis of the maxillary sinus wall compared to patients with class IV and V mutations.
CONCLUSION: These data suggest more severe sinonasal disease in patients with class I-III mutations compared to patients with class IV-V mutations.
© 2013.

Entities:  

Keywords:  Cystic fibrosis; Genotype; Nasal polyps; Rhinosinusitis

Mesh:

Substances:

Year:  2013        PMID: 24210900     DOI: 10.1016/j.jcf.2013.10.011

Source DB:  PubMed          Journal:  J Cyst Fibros        ISSN: 1569-1993            Impact factor:   5.482


  13 in total

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4.  Clinical predictors of cystic fibrosis chronic rhinosinusitis severity.

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Review 5.  Precision Genomic Medicine in Cystic Fibrosis.

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6.  Sinus biofilms in patients with cystic fibrosis: is adjusted eradication therapy needed?

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7.  Intranasal micro-optical coherence tomography imaging for cystic fibrosis studies.

Authors:  Hui Min Leung; Susan E Birket; Chulho Hyun; Timothy N Ford; Dongyao Cui; George M Solomon; Ren-Jay Shei; Adegboyega Timothy Adewale; Andrew R Lenzie; Courtney M Fernandez-Petty; Hui Zheng; Justin H Palermo; Do-Yeon Cho; Bradford A Woodworth; Lael M Yonker; Bryan P Hurley; Steven M Rowe; Guillermo J Tearney
Journal:  Sci Transl Med       Date:  2019-08-07       Impact factor: 17.956

8.  Outcomes of endoscopic sinus surgery in adult lung transplant patients with cystic fibrosis.

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9.  Medical and Surgical Advancements in the Management of Cystic Fibrosis Chronic Rhinosinusitis.

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Review 10.  Orphan diseases of the nose and paranasal sinuses: Pathogenesis - clinic - therapy.

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