The Turkish Journal of Pediatrics 2023 , Vol 65 , Num 2
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
1 Department of Pediatric Pulmonology, Gazi University Faculty of Medicine, Ankara
2 Department of Pediatric Pulmonology, Hacettepe University Faculty of Medicine, Ankara
3 Department of Pulmonology, Dicle University Faculty of Medicine, Diyarbakır
4 Department of Pediatric Pulmonology, Dicle University Faculty of Medicine, Diyarbakır
5 Department of Pediatric Pulmonology, Necmettin Erbakan University Meram Faculty of Medicine, Konya
6 Department of Pediatric Pulmonology, İstanbul University Cerrahpaşa, Cerrahpaşa Faculty of Medicine, İstanbul
7 Department of Pediatric Pulmonology, Bezmialem Vakıf University Faculty of Medicine, İstanbul
8 Department of Pediatric Allergy and Immunology, Çukurova University Faculty of Medicine, Adana
9 Department of Pediatric Pulmonology, Ege University Faculty of Medicine, İzmir
10 Department of Pediatric Pulmonology, Akdeniz University Faculty of Medicine, Antalya
11 Department of Pediatric Allergy and Immunology, Bursa Uludağ University Faculty of Medicine, Bursa
12 Department of Chest Diseases, Hacettepe University Faculty of Medicine, Ankara
13 Department of Pediatric Pulmonology, Ankara City Hospital, Ankara
14 Division of Pediatric Pulmonology, Mersin City Training and Research Hospital, Mersin
15 Department of Pediatric Allergy and Immunology, Eskişehir Osmangazi University Faculty of Medicine, Eskişehir
16 Department of Pediatric Pulmonology, Erciyes University Faculty of Medicine, Kayseri
17 Department of Pediatric Allergy and Immunology, İstanbul University Faculty of Medicine, İstanbul
18 Department of Pediatric Allergy and Immunology, Celal Bayar University Faculty of Medicine, Manisa
19 Department of Pediatric Pulmonology, Ankara University Faculty of Medicine, Ankara
20 Department of Pediatric Allergy and Immunology, İnönü University Faculty of Medicine, Malatya
21 Department of Pediatric Pulmonology, Balıkesir University Faculty of Medicine, Balıkesir
22 Department of Chest Diseases, Ege University Faculty of Medicine, İzmir
23 Department of Pediatric Gastroenterology, Hepatology and Nutrition, Ondokuz Mayıs University Faculty of Medicine, Samsun
24 Department of Pediatric Allergy and Immunology, Fırat University Faculty of Medicine, Elazığ
25 Department of Pediatric Pulmonology, Şişli Hamidiye Etfal Research and Training Hospital, İstanbul, Türkiye
DOI : 10.24953/turkjped.2021.4930 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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