Cystic fibrosis in Turkey: First data from the national registry

Abstract

Background Cystic fibrosis (CF) care has been implemented in Turkey for a long time; however, there had been no patient registry. For this purpose, the Turkish National CF Registry was established. We present the first results of registry using data collected in 2017. Methods The data were collected using a data-entry software system, which was accessed from the internet. Demographic and annually recorded data consisted of 15 and 79 variables, respectively. Results There were 1170 patients registered from 23 centers; the estimated coverage rate was 30%. The median age at diagnosis was 1.7 years (median current age: 7.3 years); 51 (4.6%) patients were aged over 18 years. Among 293 patients who were under 3 years of age, 240 patients (81.9%) were diagnosed through newborn screening. Meconium ileus was detected in 65 (5.5%) patients. Genotyping was performed in 978 (87.4%) patients and 246 (25.2%) patients' mutations were unidentified. The most common mutation was deltaF508 with an allelic frequency of 28%, followed by N1303K (4.9%). The median FEV1% predicted was 86. Chronic colonization with Pseudomonas aeruginosa was seen in 245 patients. The most common complication was pseudo-Bartter syndrome in 120 patients. The median age of death was 13.5 years in a total of 15 patients. Conclusions Low coverage rate, lack of genotyping, unidentified mutations, and missing data of lung functions are some of our greatest challenges. Including data of all centers and reducing missing data will provide more accurate data and help to improve the CF care in Turkey in the future.

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[Cakir, Erkan; Al Shadfan, Lina M.; Yazan, Hakan] Bezmialem Univ, Fac Med, Div Pediat Pulmonol, Istanbul, Turkey.

[Sismanlar, Tugba] Dr Sami Ulus Matern & Children Training & Res Hos, Div Pediat Pulmonol, Ankara, Turkey.

[Cobanoglu, Nazan] Ankara Univ, Fac Med, Div Pediat Pulmonol, Ankara, Turkey.

[Pekcan, Sevgi; Ercan, Omur] Necmettin Erbakan Univ, Meram Med Fac, Div Pediat Pulmonol, Konya, Turkey.

[Cinel, Guzin; Tugcu, Gokcen] Ankara Childrens Hematol Oncol Training & Res Hos, Div Pediat Pulmonol, Ankara, Turkey.

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

[Sen, Hadice S.] Dicle Univ, Fac Med, Dept Pulmonol, Diyarbakir, Turkey.

[Keskin, Ozlem; Eltan, Sevgi B.] Gaziantep Univ, Fac Med, Div Pediat Allergy, Gaziantep, Turkey.

[Altintas, Derya U.; Sasihuseyinoglu, Senay] Cukurova Univ, Fac Med, Div Pediat Allergy & Immunol, Adana, Turkey.

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

[Cokugras, Haluk; Kilinc, Ayse A.] Istanbul Univ, Cerrahpasa Med Fac, Div Pediat Allergy & Pulmonol, Istanbul, Turkey.

[Gursoy, Tugba R.; Aslan, Ayse T.] Gazi Univ, Fac Med, Div Pediat Pulmonol, Ankara, Turkey.

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

[Ozdemir, Ali] Mersin City Training & Res Hosp, Div Pediat Pulmonol, Mersin, Turkey.

[Kose, Mehmet; Hangul, Melih] Erciyes Univ, Fac Med, Div Pediat Pulmonol, Kayseri, Turkey.

[Emiralioglu, Nagehan] Gaziantep Cengiz Gokcek Matern & Children Hosp, Div Pediat Pulmonol, Gaziantep, Turkey.

[Yuksel, Hasan; Yilmaz, Ozge] Celal Bayar Univ, Fac Med, Div Pediat Pulmonol Allergy & Immunol, Manisa, Turkey.

[Orhan, Fazil] Karadeniz Tech Univ, Fac Med, Div Pediat Allergy, Trabzon, Turkey.

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

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

[Tamay, Zeynep; Suleyman, Ayse] Istanbul Univ, Istanbul Fac Med, Div Pediat Allergy, Istanbul, Turkey.

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

[Bal, Cem M.] Ataturk Reg Training & Res Hosp, Div Pediat Pulmonol, Erzurum, Turkey.

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