Demographic, Clinical and Laboratory Indicators of Patients with Cystic Fibrosis Referred to Imam Hossein Children's Hospital

Abstract

Background: Cystic fibrosis (CF) is a deadly genetic disease that causes various symptoms and complications. This study aimed to evaluate the demographic, clinical and para clinical characteristics among patients with CF. Methods: This cross-sectional study was performed in Imam Hossein (AS) Children's Hospital for one year, from February 1, 2017 to February 1, 2018. About 100 patients referred to Imam Hossein (AS) Children's Hospital in Isfahan diagnosed of cystic fibrosis were included in the study, and all demographic, clinical and laboratory information of the patients was recorded. Findings: Among the CF patients in this study 58 were boys and 42 girls. The average age of the patients was 102 months and within the age range of 5-15 years. The average age of children with CF at the time of diagnosis was 15 months. Around 62 of patients in whom expiratory volume was measured by pressure in one second showed mild involvement, 28 moderate involvement and 10 severe involvement. Gastroesophageal reflux disease (GERD) with 26, nasal polyp and asthma with relative frequency of 13 and 10, respectively, were the most common complications among CF patients. Only 18 of CF patients had done genetic testing. Only one patient in the present study used Dornaz alfa. In terms of longevity, only 5 of patients were over 18 years old. Conclusion: Compared to developed countries that have newborn screening, patients with CF in this study were diagnosed late. Also, CF patients benefited less from the primary drugs that control the disease, and had a shorter lifespan. © 2022 Isfahan University of Medical Sciences(IUMS). All rights reserved.