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Cystic Fibrosis


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Inhaled Glutathione Shows Promise as Cystic Fibrosis Treatment

NEW YORK (Reuters Health) Jan 21 - Treatment with inhaled buffered reduced glutathione (GSH) safely improves small airway function and other clinical parameters in patients with cystic fibrosis (CF), results of a pilot study indicate. According to a related editorial, the benefits of this therapy could extend beyond CF.

Much CF research has focused on the altered chloride efflux exhibited by pneumocytes, the report in the January issue of Chest notes. However, GSH efflux is also impaired and there is good evidence to suggest that the lack of GSH in lung fluid plays a key role in the chronic inflammation and infection that occurs with CF. Therefore, restoring GSH levels could be of therapeutic benefit.

Previous studies have investigated inhaled GSH as a treatment for CF. The current study is different from the others in that it used a placebo control and involved a higher daily dose of GSH over a longer treatment period.

In the study, 19 patients with CF were randomized to receive inhaled GSH, at a dose of about 66 mg/kg of body weight, or placebo for 8 weeks. The primary outcomes involved changes in pulmonary function, while the secondary outcomes focused largely on symptoms.

GSH-treated patients experienced an average increase in peak flow of 33.7 L/min, whereas the control group experienced a drop of 6.5 L/min (p = 0.04), lead author Dr. Clark Bishop, from Utah Valley Regional Medical Center in Provo, and colleagues note.

On 11 of the 13 primary and secondary outcomes, the GSH group had a better result than the control group. Moreover, when asked to rate their condition on a 5-point scale, a significant improvement was observed in the GSH group compared with controls (p = 0.004).

Lastly, GSH therapy was well tolerated and the frequency and nature of adverse effects was comparable to that seen in the control group.

In a related editorial, Dr. Brian J. Day, from the National Jewish Medical and Research Center in Denver, Colorado, comments that with three small studies showing a benefit for GSH therapy in CF, "it seems clear that the next logical step is a large multicenter clinical trial." However, he notes that because GSH deficiency is found in a number of lung diseases, GSH therapy may have broader implications than just CF.

Chest 2005;127:12-14,308-317.

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