Pediatric patients with cystic fibrosis with the homozygous F508del genotype in the U.S. had better lung function than children in the U.K., data published in Thorax shows.
“Previous international comparisons of outcomes in people with cystic fibrosis have highlighted the impact of different health care practices and approaches to treatment and have contributed to improvements in care for people with cystic fibrosis,” Daniela K. Schlüter,PhD, applied mathematician/statistician in the department of public health, policy and systems at the University of Liverpool, U.K., and colleagues wrote.
For the current comparative longitudinal analysis, researchers evaluated cohorts of children with cystic fibrosis aged 6 to 18 years with the homozygous F508del genotype. All were enrolled in the U.S. Cystic Fibrosis Foundation Patient Registry (n = 9,463) or the UK Cystic Fibrosis Registry (n = 3,055). Researchers compared early growth and nutrition — including height, weight and BMI — as well as infections and treatments between children in the U.S. and the U.K.
U.S. children had higher lung function when first measured at age 6 years compared with U.K. children, according to the results. These lung function measurements were sustained throughout childhood. Lung function declined faster in the U.K., at –1.61 per year compared with –1.41 in the U.S. U.K. children also lost an average of an additional 0.2 percentage points in FEV1 percent predicted per year compared with U.S. children.
