The success of the Cystic Fibrosis Registry of Turkey for improvement of

Abstract

Background Cystic fibrosis (CF) registries play an essential role in improving disease outcomes of people with CF. This study aimed to evaluate the association of newly established CF registry system in Turkey on follow-up, clinical, growth, treatment, and complications of people with this disease. Methods Age at diagnosis, current age, sex, z-scores of weight, height and body mass index (BMI), neonatal screening results, pulmonary function tests, history of meconium ileus, medications, presence of microorganisms, and follow-up were evaluated and compared to data of people with CF represented in both 2017 and 2019 registry data. Results There were 1170 people with CF in 2017 and 1637 in 2019 CF registry. Eight hundred and fourteen people were registered in both 2017 and 2019 of whom z-scores of heights and BMI were significantly higher in 2019 (p = 0.002, p =0.039, respectively). Inhaled hypertonic saline, bronchodilator, and azithromycin usages were significantly higher in 2019 (p =0.001, p = 0.001, p = 0.003, respectively). The percent predicted of forced expiratory volume in 1 sec and forced vital capacity were similar in 2017 and 2019 (88% and 89.5%, p = 0.248 and 84.5% and 87%, p =0.332, respectively). Liver diseases and osteoporosis were significantly higher, and pseudo-Bartter syndrome (PBS) was significantly lower in 2019 (p = 0.011, p = 0.001, p = 0.001, respectively). Conclusions The z-scores of height and BMI were higher, the use of medications that protect and improve lung functions was higher and incidence of PBS was lower in 2019. It was predicted that registry system increased the care of people with CF regarding their follow-up. The widespread use of national CF registry system across the country may be beneficial for the follow-up of people with CF.

C1 [Asfuroglu, Pelin; Eyuboglu, Tugba Sismanlar; Aslan, Ayse Tana; Gursoy, Tugba Ramasli] Gazi Univ, Fac Med, Dept Pediat Pulmonol, TR-06560 Ankara, Turkey.

[Emiralioglu, Nagehan; Yalcin, Ebru; Kiper, Nural; Ozcelik, Ugur; Dogru, Deniz] Hacettepe Univ, Fac Med, Dept Pediat Pulmonol, Ankara, Turkey.

[Sen, Velat] Dicle Univ, Fac Med, Dept Pediat Pulmonol, Diyarbakir, Turkey.

[Sen, Hadice Selimoglu] Dicle Univ, Fac Med, Dept Chest Dis, Diyarbakir, Turkey.

[Altintas, Derya Ufuk; Ozcan, Dilek] Cukurova Univ, Fac Med, Dept Pediat Allergy & Immunol, Adana, Turkey.

[Kilinc, Ayse Ayzit; Cokugras, Haluk; Baskan, Azer Kilic] Istanbul Univ, Cerrahpasa Med Fac, Dept Pediat Pulmonol, Istanbul, Turkey.

[Yazan, Hakan; Cakir, Erkan] Bezmialem Univ, Fac Med, Dept Pediat Pulmonol, Istanbul, Turkey.

[Erenberk, Ufuk] Bezmialem Univ, Fac Med, Dept Pediat, Istanbul, Turkey.

[Dogan, Guzide] Bezmialem Univ, Fac Med, Dept Pediat Gastroenterol, Istanbul, Turkey.

[Unal, Gokcen; Yilmaz, Asli Imran; Pekcan, Sevgi] Necmettin Erbakan Univ, Meram Med Fac, Dept Pediat Pulmonol, Konya, Turkey.

[Keskin, Ozlem; Arik, Elif; Kucukosmanoglu, Ercan] Gaziantep Univ, Fac Med, Dept Pediat Allergy & Immunol, Gaziantep, Turkey.

[Irmak, Ilim; Damadoglu, Ebru] Hacettepe Univ, Fac Med, Dept Chest Dis, Ankara, Turkey.

[Ozturk, Gokcen Kartal; Gulen, Figen] Ege Univ, Fac Med, Dept Pediat Pulmonol, Izmir, Turkey.

[Basaran, Abdurrahman Erdem; Bingol, Aysen] Akdeniz Univ, Fac Med, Dept Pediat Pulmonol Allergy & Immunol, Antalya, Turkey.

[Cekic, Sukru; Sapan, Nihat] Bursa Uludag Univ, Fac Med, Dept Pediat Allergy & Immunol, Bursa, Turkey.

[Kilic, Gonca] Osmangazi Univ, Fac Med, Dept Pediat Metab & Nutr, Eskisehir, Turkey.

[Harmanci, Koray] Osmangazi Univ, Fac Med, Dept Pediat Allergy & Immunol, Eskisehir, Turkey.

[Kose, Mehmet] Erciyes Univ, Fac Med, Dept Pediat Pulmonol, Kayseri, Turkey.

[Ozdemir, Ali] Minist Hlth, Dept Pediat Pulmonol, Mersin City Hosp, Mersin, Turkey.

[Tugcu, Gokcen Dilsa; Polat, Sanem Eryilmaz; Cinel, Guzin] Minist Hlth, Dept Pediat Pulmonol, Ankara City Hosp, Ankara, Turkey.

[Hangul, Melih] Gaziantep Cengiz Gokcek Matern & Childrens Hosp, Dept Pediat Pulmonol, Gaziantep, Turkey.

[Ozcan, Gizem; Cobanoglu, Nazan] Ankara Univ, Fac Med, Dept Pediat Pulmonol, Ankara, Turkey.

[Aydin, Zeynep Gokce Gayretli] Karadeniz Tech Univ, Fac Med, Dept Pediat Infect Dis, Trabzon, Turkey.

[Yuksel, Hasan] Celal Bayar Univ, Fac Med, Dept Pediat Allergy & Immunol, Manisa, Turkey.

[Topal, Erdem] Inonu Univ, Fac Med, Dept Pediat Allergy, Malatya, Turkey.

[Ozdogan, Sebnem] Sisli Hamidiye Etfal Res & Training Hosp, Dept Pediat Pulmonol, Istanbul, Turkey.

[Caltepe, Gonul] Ondokuz Mayis Univ, Fac Med, Dept Pediat Gastroenterol Hepatol & Nutr, Samsun, Turkey.

[Suleyman, Ayse] Istanbul Univ, Istanbul Fac Med, Dept Pediat Allergy & Immunol, Istanbul, Turkey.

[Can, Demet] Balikesir Univ, Fac Med, Dept Pediat Pulmonol, Balikesir, Turkey.

[Ekren, Pervin Korkmaz] Ege Univ, Fac Med, Dept Chest Dis, Izmir, Turkey.

[Bal, Cem Murat] Doctor Lutfi Kirdar Kartal Training & Res Hosp, Dept Pediat Pulmonol, Istanbul, Turkey.

[Kilic, Mehmet] Firat Univ, Fac Med, Dept Pediat Allergy & Immunol, Elazig, Turkey.