First Cystic Fibrosis Patient Registry Annual Data Report - Cystic Fibrosis Foundation of Iran

  • Asghar Aghamohammadi Research Center for Immunodeficiencies, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran.
  • Majid Keivanfar Department of Pediatric Pulmonary and Sleep Medicine, Imam Hossein Children's Hospital, Isfahan University of Medical Sciences, Isfahan, Iran. AND Child Growth and Development Research Center, Research Institute for Primordial Prevention of Non-Communicable Disease, Imam Hossein Children’s Hospital, Isfahan University of Medical Sciences, Isfahan, Iran.
  • Safoura Navaei Pediatric Respiratory and Sleep Medicine Research Center, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran.
  • Rohola Shirzadi Pediatric Respiratory and Sleep Medicine Research Center, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran.
  • Farzad Masiha Department of Pediatrics, Mazandaran University of Medical Sciences, Sari, Iran.
  • Zahra Allameh Child Growth and Development Research Center, Research Institute for Primordial Prevention of Non-Communicable Disease, Imam Hossein Children’s Hospital, Isfahan University of Medical Sciences, Isfahan, Iran.
  • Amin Heydari Research Center for Immunodeficiencies, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran.
  • Solat Eslami Dietary Supplements and Probiotic Research Center, Alborz University of Medical Sciences, Karaj, Iran.
  • Gholamreza Azizi Non-Communicable Diseases Research Center, Alborz University of Medical Sciences, Karaj, Iran.
  • Mohsen Reisi Department of Pediatric Pulmonary and Sleep Medicine, Imam Hossein Children's Hospital, Isfahan University of Medical Sciences, Isfahan, Iran. AND Child Growth and Development Research Center, Research Institute for Primordial Prevention of Non-Communicable Disease, Imam Hossein Children’s Hospital, Isfahan University of Medical Sciences, Isfahan, Iran.
  • Mohammadreza Modaresi Pediatric Respiratory and Sleep Medicine Research Center, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran.
Keywords: Cystic fibrosis, Demographic factors, Clinical markers, Laboratory indices

Abstract

Cystic fibrosis (CF), as a fatal genetic condition, is associated with high morbidity and mortality rates. In Iran, limited studies exist on this disease. This study aims to compare the demographic, clinical and paraclinical data of CF patients. This cross-sectional study was conducted in 2014-2015 on 174 CF patients referred to the Tehran Children Medical Center hospital, which is the main referral center for CF. For each patient, the forced  expiratory volume in one second (FEV1) was measured and the comparative demographic, clinical and laboratory data of patients were recorded. Overall, 59% of studied patients were boys (n=102) and 41% were girls (n=72). The mean patient age (and standard deviations) was 7.1 ±5.7 years, with a range of 10 days to 28 years. In 67% of cases, the disease was diagnosed before their first birthday. The patients in this study were classified based on the FEV1 into mild (62%), moderate (33%) and sever (5%), indicating the degree of pulmonary complications. Cultures of respiratory secretions were positive for Pseudomonas aeruginosa and Staphylococcus aureus, in 23% and 16% of cases, respectively. In total, 61% of patients (n=83) were assigned to receive oral azithromycin for prophylaxis. Gastroesophageal reflux (reflux) was the most common gastrointestinal complication (35%), Regarding the complex nature of CF and the necessity of constant monitoring of patients during the life-span, the comparative demographic, clinical and laboratory analysis of patients and registering and standardization of patients’ data, can be a major step in the better understanding of the disease, and thereby increasing the quality of life and life expectancy in the affected population.

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Published
2019-02-13
How to Cite
1.
Aghamohammadi A, Keivanfar M, Navaei S, Shirzadi R, Masiha F, Allameh Z, Heydari A, Eslami S, Azizi G, Reisi M, Modaresi M. First Cystic Fibrosis Patient Registry Annual Data Report - Cystic Fibrosis Foundation of Iran. Acta Med Iran. 57(1):33-41.
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Articles