A 6-year Evaluation of Cystic Fibrosis
Nooshin Baghaei, Soheila Khalilzadeh, Mohammad Reza Boloursaz, Shahin Hakimi, Amir Ali Khodayari, Ali Akbar Velayati
National Research Institute of Tuberculosis and Lung Disease, Shahid Beheshti University of Medical Sciences and Health Services, Iran
Abstract

Background and objectives: Cystic fibrosis (CF) is the most prevalent autosomal recessive hereditary disease in children. The mortality rate of children with CF has been reported to be higher in developing countries compared to developed countries.

This study was conducted to evaluate the clinical, paraclinical and radiological status of CF patients hospitalized in the pediatric ward of Masih Daneshvari Hospital. Complications were compared in patients who had followed their prophylactic treatment with those who did not.

Materials and Methods: Medical records of patients hospitalized at the Masih Daneshvari Hospital with a diagnosis of CF during 2000-2005 were evaluated. These patients were divided into two groups: 1) patients who had followed the prophylactic treatment regularly and 2) those who had not followed prophylactic treatment on a regular basis.

Results: A total of 32 children with CF were evaluated. Forty eight percent were males and 52% were females. The mean age of the patients was 14.46± years. The most common symptoms were chronic productive cough, developmental delay and gastrointestinal complications. The most prevalent radiological finding was bronchiectasis which was seen in 48% of the cases. The prevalence of respiratory infections and bronchiectasis was significantly higher in the second group as compared to the first (P=0.03). Five patients had passed away due to the respiratory complications of CF. The mean age of patients at the time of death was 16.5 ± 7.6 years.

Discussion and Conclusion: Occurrence of recurrent respiratory infections is one of the most distressing complications of CF. Considering the results of this study, implementing proper prophylactic treatment and training patients regarding the regular use of prescribed drugs and performing respiratory physiotherapy can be effective in decreasing disease complications.
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