The Evaluation of the Genotype and Phenotype Relationship in Patients With Nasal Polyps in Cystic Fibrosis Registry of Turkey
Makaleler > Tam Makale · 2026
Yazarlar: Handan Kekeç, Tuğba Şişmanlar Eyüboğlu, Ayşe Tana Aslan, Volkan Medeni, Ebru Yalçın, Ömer Faruk İpek, Tugçe Kantemir, Bahar Ece Tokdemir, Fatma Nur Ayman, Figen GÜLEN, Atacan Ogutcu, Abdurrahman Erdem Başaran, Ayşen Bingol, Nazan Cobanoglu, Yakup Canıtez, Cansu Altıntaş, Doruk Gul, Gamze Akca Dinc, Beste Özsezen, Tuğba Ramaslı Gürsoy, Ali Özdemir, Demet Polat Yuluğ, Koray Harmancı, Hasan Yüksel, Gülben Özgül Postuk, Melih Hangul, Erdi Çağrı Dağdelen, Pelin Asfuroğlu, Mehmet Kılıç, Velat Şen, Hatice Selimoğlu Şen, Suat Savaş, Ayşe Ayzıt Kılınç, Ciğdem Korkmaz, Berrak Oztosun, Mehmet Köse, Ali Ersoy, Ozlem Keskin, Gaye İnal, Derya Ufuk Altıntaş, Mahir Serbes, Gönül Caltepe, Erdem Topal, Mina Hızal, Eylül Pınar Çakır, Nilay Baş İkizoğlu, Halime Nayır Büyüksahin, Fazılcan Zirek, Ece Ocak, Nagehan Emiralioğlu, Güzin Cinel, Sevgi Pekcan, Erkan Çakır, Uğur Özçelik, Deniz Doğru
Pediatric Pulmonology, Uluslararası, Wos, 2026, cilt 61, ss e71608-e71608
DOI: 10.1002/ppul.71608
Özet
INTRODUCTION: Cystic fibrosis (CF) is a complex genetic disorder characterized by significant clinical heterogeneity. We aimed to investigate the genotype-phenotype relationship of nasal polyps (NP) in CF Registry of Turkey (CFRT). METHODS: The study included all children registered in the CFRT in 2023, divided into two groups based on NP presentation. Clinical and demographic data were compared. The Sinus-Nasal Quality of Life (QoL) Survey-5(SN-5) evaluated patients with NP history. RESULTS: A total of 2258 patients were registered, with 115 (5.1%) having NP and 2143 without NP. Age was higher in the NP group (p < 0.001). The first and second sweat tests were higher in the NP group (p < 0.05). Median FEV1% was 89 (31-133) for the NP group and 85 (15-142) for those without NP (p = 0.015). Chronic colonization of MSSA and MRSA, CF-related liver disease, and CF-related diabetes were also more common in NP (p < 0.05). No differences were detected in pulmonary exacerbations (p > 0.05). In the NP group, severe genotype and F508del were more frequent (p < 0.05). Elexacaftor/tezacaftor/ivacaftor use was more common in NP (p = 0.003). A weak negative correlation was found between age and the SN-5 score (p = 0.020, r = -0.231), as well as a weak positive correlation between age and the Visual Analog Scale (VAS) (p = 0.001, r = 0.322). CONCLUSION: Although uncommon, NP is associated with more severe clinical and genetic characteristics in CF. These findings underscore the importance of integrating upper airway evaluation into CF management and highlight the potential for age-related adaptation or improved symptom perception in older patients.