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Pediatric pulmonology · 2023

Patients with cystic fibrosis who could not receive the CFTR modulator treatment: What did they lose in 1 year?

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Uytun Salih, Cinel Güzin, Eryılmaz Polat Sanem, Özkan Tabakçı Satı, Kiper Nural, Yalçın Ebru, Ademhan Tural Dilber, Özsezen Beste, Şen Velat, Selimoğlu Şen Hadice, Ufuk Altıntaş Derya, Çokuğraş Haluk, Kılınç Ayşe Ayzıt, Başkan Azer Kılıç, Yazan Hakan, Çollak Abdulhamit, Uzuner Selçuk, Ünal Gökçen, Yılmaz Aslı İmran, Çağlar Hanife Tuğçe, Damadoğlu Ebru, Irmak İlim, Demir Esen, Kartal Öztürk Gökçen, Bingöl Ayşen, Başaran Erdem, Sapan Nihat, Canıtez Yakup, Tana Aslan Ayşe, Asfuroğlu Pelin, Harmancı Koray, Köse Mehmet, Hangül Melih, Özdemir Ali, Çobanoğlu Nazan, Özcan Gizem, Keskin Özlem, Yüksel Hasan, Özdoğan Şebnem, Topal Erdem, Çaltepe Gönül, Can Demet, Korkmaz Ekren Pervin, Kılıç Mehmet, Emiralioğlu Nagehan, Şişmanlar Eyüboğlu Tuğba, Pekcan Sevgi, Çakır Erkan, Özçelik Uğur, Doğru Deniz

Аннотация

BACKGROUND: Cystic fibrosis (CF) is an autosomal recessive disorder caused by CF transmembrane conductance regulator (CFTR) genetic variants. CFTR modulators improve pulmonary function and reduce respiratory infections in CF. This study investigated the clinical and laboratory follow-up parameters over 1 year in patients with CF who could not receive this treatment.

METHODS: This retrospective cohort study included 2018 and 2019 CF patient data from the CF registry of Turkey. Demographic and clinical characteristics of 294 patients were assessed, who had modulator treatment indications in 2018 but could not reach the treatment.

RESULTS: In 2019, patients younger than 18 years had significantly lower BMI z-scores than in 2018. During the 1-year follow-up, forced expiratory volumes (FEV1) and FEV1 z-scores a trend toward a decrease. In 2019, chronic Staphylococcus aureus colonization, inhaled antipseudomonal antibiotic use for more than 3 months, oral nutritional supplement requirements, and oxygen support need increased.

CONCLUSIONS: Patients who had indications for modulator treatments but were unable to obtain them worsened even after a year of follow-up. This study emphasized the importance of using modulator treatments for patients with CF in our country, as well as in many countries worldwide.

Предметные рубрики MeSH

ИП Урсолов Владлен Сергеевич

ИНН 500118482352 · ОГРНИП 314500117700049

Реквизиты и контакты