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Improvement in clinical markers in CF patients using a
reduced glutathione regimen: an uncontrolled observational study
Visca A, Bishop CT, Hilton SC, Hudson VM
J Cyst Fibros, 2008 May 20; Epub ahead on print
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ABSTRACT
CFTR mutation, which causes cystic fibrosis (CF), has also recently been
identified as causing glutathione system dysfunction and systemic
deficiency of reduced glutathione (GSH). Such dysfunction and
deficiency regarding GSH may contribute to the pathophysiology of CF.
We followed 13 patients (age range 1-27 years) with cystic fibrosis who
were using a regimen of reduced glutathione (GSH), including oral
glutathione and inhaled buffered glutathione in an uncontrolled,
observational study. Dosage ranged from 66-148mg/kg/day in divided
doses, and the term examined was the initial 5.5 months of GSH use (45
days of incrementally adjusted dose, plus 4 months of use at full
dosage). Baseline and post-measurements of FEV 1 percent
predicted, BMI percentile, and weight percentile were noted, in addition
to bacterial status and pulmonary exacerbations. Significant
improvement in the following clinical parameters was observed: average
improvement in FEV 1 percent predicted (N=10) was 5.8 percentage points
(p<0.0001), average weight percentile (N-13) increased 8.6 points
(p<0.001), BMI percentile (N=11) improved on average 1.22 points
(p<0.001). All patients improved in FEV 1 and BMI, if measured in
their case; 12 of 13 patients improved in weight percentile.
Positive sputum cultures of bacteria in 11 patients declined from 13 to
5 (p<0.03) with sputum cultures of Pseudomonas aeruginosa becoming
negative in 4 of 5 patients previously culturing PA, including two of
three patients chronically infected with PA as determined by antibody
status. Use of a daily GSH regimen appears to be associates in CF
patients with significant improvement in lung function and weight, and a
significant decline in bacteria cultured in this uncontrolled study.
These findings bear further clinical investigation in larger,
randomized, controlled studies.
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