Clinical and laboratory characteristics of patients with cystic fibrosis in Jordan: A report on 50 patients

Backgroud Cystic fibrosis (CF) is a genetic disorder affecting multiple organ systems, with varying prevalence rates worldwide. Objectives To understand the clinical and laboratory profile of people with CF (pwCF) in Jordan. Methods This descriptive study of 50 pwCF undergoing treatment at the CF clinic at Prince Hamza Hospital in Amman, Jordan, from 2016 to 2024. Results The average age of diagnosis was 4.2 years. Consanguinity was noted in 88% of the patients. Respiratory complications like chronic cough (70%) and digital clubbing (72%) were common, along with gastrointestinal and endocrine problems. Pulmonary function tests indicated a mean forced expiratory volume (FEV1) of 70.6% of the predicted value. Genetic testing revealed that the most prevalent mutations were G85E (16%), F508del (12%), and CFTRdel19-21 (10%). Microbial analysis revealed Pseudomonas aeruginosa as the predominant pathogen in 50% of sputum cultures. Bronchiectasis was identified in 56% of pwCF. The study also assessed medical management and patient adherence, finding that 58.8% of the pwCF attended the clinic regularly. Compliance with chest physiotherapy was low (5.6%). Conclusion This study contributes valuable insights into the clinical profile of patients with CF in Jordan, highlighting the need for improved early diagnosis, consistent care, and better access to therapies. The findings underscore the challenges of managing CF in a low-resource setting.