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. 2023;65(2):257-268.
doi: 10.24953/turkjped.2021.4930.

Clinical characteristics of patients requiring lung transplantation referral in national cystic fibrosis registry data

Zeynep Reyhan Onay  1 , Tuğba Şismanlar Eyüboğlu  1 , Ayşe Tana Aslan  1 , Tuğba Ramaslı Gürsoy  1 , Ebru Yalçın  2 , Nural Kiper  2 , Nagehan Emiralioğlu  2 , Hadice Selimoğlu Şen  3 , Velat Şen  4 , Gökçen Ünal  5 , Aslı İmran Yılmaz  5 , Ayşe Ayzıt Kılınç  6 , Haluk Çokuğraş  6 , Azer Kılıç Başkan  6 , Hakan Yazan  7 , Abdulhamit Çollak  7 , Selçuk Uzuner  7 , Ayşe Şenay Şasihüseyinoğlu  8 , Dilek Özcan  8 , Derya Ufuk Altıntaş  8 , Gökçen Kartal Öztürk  9 , Esen Demir  9 , Ayşen Bingöl  10 , Erdem Başaran  10 , Şükrü Çekiç  11 , Nihat Sapan  11 , İlim Irmak  12 , Ebru Damadoğlu  12 , Gökçen Dilşa Tuğcu  13 , Sanem Eryılmaz Polat  13 , Ali Özdemir  14 , Koray Harmancı  15 , Gonca Kılıç  15 , Melih Hangül  16 , Mehmet Köse  16 , Zeynep Tamay  17 , Hasan Yüksel  18 , Gizem Özcan  19 , Erdem Topal  20 , Demet Can  21 , Pervin Korkmaz  22 , Gönül Çaltepe  23 , Mehmet Kılıç  24 , Şebnem Özdoğan  25 , Erkan Çakır  7 , Nazan Çobanoğlu  19 , Sevgi Pekcan  5 , Güzin Cinel  13 , Uğur Özçelik  2 , Deniz Doğru  2
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Free article

Clinical characteristics of patients requiring lung transplantation referral in national cystic fibrosis registry data

Zeynep Reyhan Onay et al. Turk J Pediatr. 2023.
Free article

Abstract

Background: We aimed to determine the number of cystic fibrosis (CF) patients recorded in the Cystic Fibrosis Registry of Türkiye (CFRT) who were in need of lung transplantation (LT) referral and examine clinical differences between patients who were LT candidates due to rapid forced expiratory volume in one second (FEV₁) decline and LT candidates without rapid FEV₁ decline in the last year to identify a preventable cause in patients with such rapid FEV₁ decline.

Methods: All CF patients recorded in the CFRT in 2018 were evaluated in terms of LT. Patients were divided into those with FEV₁ below 50% and in need of LT due to a decrease of 20% or more in the previous year (Group 1) and those who did not have FEV₁ decline of more than 20% in the previous year but had other indications for LT (Group 2). Demographic and clinical features were compared between the two groups.

Results: Of 1488 patients registered in CFRT, 58 had a need for LT. Twenty patients were included in Group 1 and others in Group 2. Our findings did not reveal any significant variations in treatment, chronic infection status, or complications between the two groups. The average weight z-score was significantly higher in Group 1. Positive correlations were detected between weight z-score and FEV₁ in 2017 in Group 1 and between FEV₁ values in 2017 and 2018 in Group 2.

Conclusions: There appears to be a relationship between the nutritional status and weight z-scores of CF patients and pulmonary function, which may indirectly affect the need for lung transplantation referral.

Keywords: cystic fibrosis; lung transplantation; registry.

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