Cystic fibrosis in Austria

Area of Science:

• Medical Research
• Public Health
• Genetics

Background:

• National-level data on cystic fibrosis (CF) patient frequency and clinical status were previously unavailable for Austria.
• Registry data are crucial for understanding CF natural history, therapy benchmarking, and clinical study recruitment.

Purpose of the Study:

• To collect and analyze comprehensive data on Austrian CF patients treated in 2014.
• To establish a national benchmark for CF patient care and public health analysis in Austria.

Main Methods:

• Data were collected from 13 Austrian CF outpatient clinics using the European CF registry and individual searches.
• A total of 773 CF patients were analyzed, with a median age of 18.9 years.

Main Results:

• The most common genotype was homozygous F508del (48.8%). Mean age at diagnosis was 27 days.
• 59% of patients had Forced Expiratory Volume in 1 second (FEV1) <80%, with a mean annual decline of 1.9% between ages 6-18.
• Prevalence of Pseudomonas aeruginosa colonization varied (12-69% in adults, 0-16% in children). Other findings included Burkholderia cepacia complex presence (3.8%), diabetes (14.5%), and liver involvement (36.3%).

Conclusions:

• This study provides the first comprehensive overview of the CF patient population in Austria.
• The collected data serve as a foundation for clinical benchmarking and public health evaluations of CF care in Austria.