Lung Function Initially Appears Normal in Cystic Fibrosis Patients

The results of a new study show that in the first few months of life, lung function is normal in patients with cystic fibrosis, suggesting that interventions designed to preserve function should be implemented during this time.

"We might be able to stop some of the lung function reduction we're identifying in the first months of life," lead author Dr. Sarath Ranganathan, from the Royal Children's Hospital in Melbourne, Australia, said in a statement. "We don't know what's going to work, but we have to target those patients in the first six months of life if we're going to be effective."

In the new study, Dr. Ranganathan's team used standard tests to assess lung function in 68 infants with cystic fibrosis and in 49 healthy controls. The cystic fibrosis infants, who ranged in age from 6 weeks to 30 month, also underwent bronchoalveolar lavage to assess lung infection and inflammation.

The study results are reported in the second December issue of the American Journal of Respiratory and Critical Care Medicine.

As the age of cystic fibrosis infants increased, the deficit in forced expiratory volume in 0.5 seconds also rose. Below 6 months of age, however, the mean value was not significantly different from that seen in healthy controls. Similar results were seen with forced vital capacity and forced expiratory flows at 75% of exhaled vital capacity.

The lung function results could not be explained by infection and inflammation in the lavage samples, the authors note.

"These findings," the investigators conclude, "have important implications for our understanding of the pathophysiologic process of early cystic fibrosis lung disease and for informing future therapeutic trials."

Source : http://www.medscape.com/viewarticle/584867