The Evaluation of the Genotype and Phenotype Relationship in Patients With Nasal Polyps in Cystic Fibrosis Registry of Turkey
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.
Authors
- Suat Savaş (ORCID: https://orcid.org/0000-0002-2733-7194)
- Bahar Ece Tokdemir (ORCID: https://orcid.org/0000-0002-6516-8547)
- Ayse Ayzıt KILINÇ
- Mahir Serbes (ORCID: https://orcid.org/0000-0001-6422-2639)
- Erdi Çağrı Dağdelen (ORCID: https://orcid.org/0000-0001-6718-0497)
- Halime Nayır Büyükşahin (ORCID: https://orcid.org/0000-0002-6909-7993)
- Mina Hızal (ORCID: https://orcid.org/0000-0002-6922-4948)
- Derya Ufuk Altıntaş (ORCID: https://orcid.org/0000-0003-2090-5248)
- Ece Ocak (ORCID: https://orcid.org/0000-0002-4466-0620)
- Demet Polat Yuluğ (ORCID: https://orcid.org/0000-0002-8122-4457)
- Figen Gülen (ORCID: https://orcid.org/0000-0002-5431-3913)
- Doruk Gül (ORCID: https://orcid.org/0000-0001-7151-4138)
- Güzin Cinel (ORCID: https://orcid.org/0000-0002-6209-196X)
- Tuğba Ramaslı Gürsoy (ORCID: https://orcid.org/0000-0002-7064-7585)
- Fatma Nur Ayman (ORCID: https://orcid.org/0000-0002-8252-7775)
- Berrak Öztosun (ORCID: https://orcid.org/0000-0001-5649-8045)
- Koray Harmancı (ORCID: https://orcid.org/0000-0002-8494-648X)
- Pelin Asfuroğlu (ORCID: https://orcid.org/0000-0002-9575-3982)
- S. Pekcan
- Abdurrahman Erdem Başaran (ORCID: https://orcid.org/0000-0002-9092-6936)
- E. Çakır
- Gönül Çaltepe (ORCID: https://orcid.org/0000-0001-8525-6352)
- Ayşen Bingöl (ORCID: https://orcid.org/0000-0002-0886-3332)
- Gamze Akça Dinç
- Fazılcan Zirek (ORCID: https://orcid.org/0000-0002-2146-9284)
- Beste Özsezen (ORCID: https://orcid.org/0000-0002-0052-8361)
- Handan Kekeç (ORCID: https://orcid.org/0000-0001-8770-0939)
- Yakup Canıtez
- Gülben Özgül Postuk (ORCID: https://orcid.org/0000-0002-4363-1254)
- Omer Faruk Ipek (ORCID: https://orcid.org/0009-0005-1126-5965)
- Hatice Selimoğlu Şen
- Mehmet Rıfat Köse
- Eylül Pınar Çakır (ORCID: https://orcid.org/0000-0003-2067-3256)
- Gaye İnal (ORCID: https://orcid.org/0000-0002-1019-7332)
- Atacan ÖĞÜTÇÜ
- Nazan Çobanoğlu
- Ayse Tana Aslan
- NİLAY BAŞ İKİZOĞLU
- Ozlem Keskin
- Tugçe Kantemir
- Uğur Özçelik
- Mehmet Kılıç
- Nagehan Emiralioğlu
- Velat Şen
- Erdem Topal
- Hasan Yüksel
- Melih Hangul
- Ebru Yalçın
- Cansu Altıntaş
- Deniz Doğru
- Ali Ersoy
- Volkan Medeni
- Tuğba Şişmanlar Eyüboğlu
- Ciğdem Korkmaz
- Ali Özdemir
Institutions
- Dicle University (TR)
- Ministry of Health (TR)
- Bursa Uludağ Üni̇versi̇tesi̇ (TR)
- Izmir University (TR)
- Ankara University (TR)
- Karadeniz Technical University (TR)
- Manisa Celal Bayar University (TR)
- Bilkent University (TR)
- Bursa Technical University (TR)
- Dokuz Eylül University (TR)
- Akdeniz University (TR)
- Inonu University (TR)
- Mardin Artuklu University (TR)
- Ondokuz Mayıs University (TR)
- Ege University (TR)
- Gaziantep Children's Hospital (TR)
- ENT and Allergy (US)
- Necmettin Erbakan University (TR)
- Istanbul University-Cerrahpaşa (TR)
- Samsun University (TR)
- Istinye University (TR)
- Education Training And Research (US)
- Başkent University Hospital (TR)
- Malatya Devlet Hastanesi (TR)
- S.B.Ü. Bursa Yüksek İhtisas Eğitim ve Araştırma Hastanesi (TR)
- Eskişehir Osmangazi University (TR)
- Mersin Üniversitesi (TR)
- Cukurova University (TR)
- Gaziantep University (TR)
- Hacettepe University (TR)
- Istanbul University (TR)
- Erciyes University (TR)
- Gazi University (TR)
Publication Details
- Journal
- Pediatric Pulmonology
- Published
- 2026-04-01
- DOI
- https://doi.org/10.1002/ppul.71608
- Primary Topic
- Cystic Fibrosis Research Advances
- Type
- article
- Field-Weighted Citation Impact
- 0.00