Introduction: The establishment of cystic fibrosis (CF) databases allows caregivers to compare different approaches and improve CF care. The longitudinal data allows the understanding of trends in CF clinical course. We present the 9 years data of the Israeli CF Patient Registry.
Methods: Epidemiological, genetic and clinical data from all CF patients were entered into database registry during the years 2005-13.
Results: 529 patients were followed in 2013, 58% of them adults (>18 yrs), compared to 498 in 2005, 47% adults (p=0.048). Median FEV1 improved from 79% predicted in 2008 to 82.5% predicted in 2013 (p=0.015). Despite the improved outcome the prevalence of CF decreased from 1:14,000 in 2005 to 1:15,370 in 2013 (p=0.004), probably due to less CF births.
The median age at diagnosis was 0.7y (0-65y), meconium ileus was the presenting symptom in 14%. W1282X and deltaF508 were the most common CFTR mutations (23% each).
Chronic growth of P.aeruginosa in sputum was observed in 40% of patients compared to 32% in 2005 (p=0.043). Staphyloccocal colonization increased from 14% to 34% in these years (p<0.05).
Median BMI-SDS in CF children was -0.1. CF adults had median BMI of 22.5 (females-20.8, males-22.9) compared to 21.2 in 2007 (p<0.05).
CF related diabetes was reported in 11% in 2005 and 19% in 2013 (p=0.001).
Summary: CF patients in 2013 are older, having better lung function and nutritional status, possibly due to improved care but with higher prevalence of bacterial colonization.
