| خلاصه مقاله | The Iranian Cystic Fibrosis Registry (ICFR) is a centralized database of CF patients in North-western of Iran that collects information on the demographic, clinical data, laboratory resultsand Fallow upping patients. Information is collected on hospitalizations and outpatients CF patients in the Educational and Treatment Children's Hospital from 2001 to 2018 with a goal to support research, education and presentation services on CF and develop cooperation with other provinces in Iran. We report here on the characteristics of patients over 17 years in Tabriz University of Medical Sciences, Iran. Of 588 CF patients, 348 were male and 240 female and 94 (22.6%) patients had died. The median age at onset, age at diagnosis, age of death and age for study were 3, 9, 0.4 and 7.8 years, respectively. The frequency of Chronicsinopulmonary disease, Gastrointestinal and nutritional abnormalities, Salt loss syndromes and Genital abnormalities were observed in 215 (51.4%), 272 (65.1%), 168 (40.2%) and 5 (1.2%) patientsat lifespan, respectively. More genotypes were heterozygote (41.9%)in compassion with homozygote (31.8%) and compoundgenotypes (17.7%) with the perdominace of the AF508/AF508 (26%) genotype among patients. All the information in the ICFR is held confidentially with Linking and disturbing capability for other medical members in Iran. ICFR Report may show an important tool to analyzeclinical and Para clinicalcharacteristicsof the disease as well as to apply for healthcare governmental programs based on health care data |