Clinical manifestation, laboratory findings, and outcome of children with cystic fibrosis over a 10-year period in South Iran

Abstract

Abstract Background Cystic fibrosis (CF) is a monogenic hereditary disease with diverse complications, which substantially reduce the quality of life and longevity of patients. With regard to the scarcity of information on epidemiological aspects of this disease in Iran, we aimed to examine the current status of pediatric CF patients in a tertiary referral center in south Iran. Results Medical records of 105 patients (51% female) with the mean age of 16.3 ± 34.4 months were reviewed. Twelve patients (13.3%) had siblings with CF. Failure to thrive and respiratory symptoms were the most common presentations. Elevated liver enzymes and steatorrhea were detected in over half of tested patients, while abnormal serum albumin level, urinalysis, positive urine cultures, and positive acute phase reactants were noticed in less than half of the patients. Mild increase in pulmonary artery blood pressure was the most common finding of echocardiography. Increased liver echogenicity was the top finding of abdominopelvic sonography. Out of 42 available patients, 19 (45.2%) died with the mean age of 54.4 ± 101.8 months. The highest rate of mortality was observed in the 2–6 years old age group (42.9%). Conclusion Increased liver echogenicity was the most common finding in hepatobiliary evaluation. Mild increase in pulmonary artery blood pressure was the most common finding of echocardiography.