Posts Tagged ‘cystic fibrosis’

The cause behind bone problems that generally accompany cystic fibrosis was traced to genetic reasons, according to a recent study by Dr Christina Haston, a researcher of the McGill University Health Centre research Institute.

This research article was published in the American Journal of Respiratory and Critical Care Medicine. The study suggested that bone frailty is caused by genetic reasons.

From Sciencedaily.com:

The medical community generally considers the bone fragility associated with cystic fibrosis to be multifactorial. It is thought to be a consequence of the mutation of the Cftr gene, the gene responsible for cystic fibrosis, of the pancreatic disease associated with cystic fibrosis and of the treatment with steroids to facilitate breathing.

The study showed that mice with a Cftr gene mutation have a bone mineral density and bone mass that are significantly lower than those of control mice. This difference occurs without the pancreatic insufficiency seen clinically and in the absence of steroid treatment.

This conclusion clearly defines cystic-fibrosis-related bone problems as an additional pathology stemming from the Cftr mutation and not as a side effect of treatment. This may have some therapeutic consequences as it opens an avenue for defining a targeted treatment in mice.

The study was funded by the Canadian Cystic Fibrosis Foundation, the Canadian Institutes for Health Research, Valorisation Recherche Quebec, and the Fonds de la Recherche en Santé du Québec.

There is no difference in terms of major disease factors among patients suffering from cystic fibrosis (CF) receiving inhaled corticosteroids and those who do not.

Disease factors consist of the number of prescribed antibiotics, lung function decline, and onset time of acute chest exacerbation or frequency of using a bronchodilator.

From News-Medical.Net:

“Oral corticosteroids slow the progression of CF lung disease, but long-term use is precluded by unacceptable side effects,” said Dr. Balfour-Lynn. “A systematic review of inhaled corticosteroid use in CF revealed 10 randomized controlled trials, with six having been published. The trials studied 293 adults and children. Although there was variable methodological quality among the studies, the conclusion was that there was ‘no evidence from existing trials to support the practice of prescribing inhaled steroids in cystic fibrosis.’”

The authors noted that 52 percent of the patients were on high-dose inhaled corticosteroids (1,000 micrograms or more per day). At those levels, the drug can lead to significant symptoms related to adrenal suppression and insufficiency. Also, among pediatric patients, slowing of linear growth has been a problem for individuals taking the drug for a year or more.

Ian M. Balfour-Lynn, M.D., F.R.C.P., of the Department of Pediatric Respiratory Medicine at Royal Brompton Hospital in London, and six associates, concluded that patients can stop making the use of inhaled corticosteroids for reducing drug burden and to reduce potential adverse side effects.