Cystic fibrosis in Ontario

Area of Science:

• Pediatrics
• Medical Genetics
• Epidemiology

Background:

• Cystic Fibrosis (CF) is a significant genetic disorder requiring accurate incidence data for public health planning.
• Understanding CF epidemiology in specific populations is crucial for resource allocation and intervention strategies.

Purpose of the Study:

• To determine the incidence of cystic fibrosis in Ontario, Canada, from 1966 to 1980.
• To analyze diagnostic timing and identify associated conditions like meconium ileus.

Main Methods:

• Utilized clinical data from the Hospital for Sick Children's CF database.
• Incorporated population statistics from the Province of Ontario.
• Analyzed records of 420 confirmed cystic fibrosis cases born between 1966 and 1980.

Main Results:

• The mean incidence of cystic fibrosis was 1 in 2,927 births.
• A decline in incidence was observed in the last five years of the study period.
• Early diagnosis was common: 60% by age 1, 74% by age 2, and 90% by age 5.
• Meconium ileus occurred in 15.7% of cases.
• Neonatal mortality was higher in males than females with CF.

Conclusions:

• The incidence of cystic fibrosis in Ontario during 1966-1980 was 1 in 2,927.
• Observed decline in later years may indicate the effectiveness of early diagnosis and genetic counseling.
• Earlier diagnosis in males compared to females was noted, alongside higher neonatal mortality in males.