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Glutathione Articles - Cystic Fibrosis
Lymphocyte Glutathione Levels in Children With Cystic
Fibrosis
Larry C. Lands, Vijaylaxmi Grey, Argyrios A.
Smountas, Violeta G. Kramer and Danielle McKenna
Chest 1999;116;201-205
DOI 10.1378/chest.116.1.201
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ABSTRACT
Objective: Lung disease
in cystic fibrosis (CF) is characterized by a neutrophilic inflammatory
response. This can lead to the production of oxidants, and to oxidative
stress in the lungs. Glutathione (GSH) represents the primary
intracellular antioxidant, and provides an important defense in the
epithelial lining fluid. Evidence suggests that lymphocyte GSH reflects
lung GSH concentrations, and so could potentially serve as a peripheral
marker of lung inflammation.
Methods: We assessed peripheral blood lymphocyte GSH concentrations
in 20 children (13 boys) with CF who were in stable condition at the time
of evaluation. Values were compared with nutritional status and lung
function parameters.
Results: Patients were 11.7 6 3.03 years old (mean 6 SD). Their
percentage of ideal body weight was 101.8 6 17.92%; FEV1, 79.5 6 19.22%
predicted; FEV1/FVC, 75.0 6 10.08%; and residual volume (RV)/total lung
capacity (TLC), 31.3 6 10.47%. For the group, the GSH concentration was
1.31 6 0.52 mmol/106 lymphocytes, which was not different from laboratory
control values. GSH values were correlated with nutritional status
(percentage of ideal body weight: r 5 0.49, p < 0.03) and the degree of
gas trapping (RV/TLC: r 5 0.50, p < 0.03), and were correlated inversely
with airflow limitation (FEV1, percent predicted: r 5 20.45, p < 0.05;
FEV1/FVC: r 5 20.48, p < 0.04), but not with age, height, or weight (p >
0.1).
Conclusions: We interpret the inverse correlation between
lymphocyte GSH concentration and lung function as a reflection of
upregulation of GSH production by lung epithelial tissue in response to
oxidative stress. We interpret the correlation between lymphocyte GSH
concentration and nutritional status as a reflection of the role of
cysteine in hepatic glutamine metabolism. Peripheral blood lymphocyte GSH
concentration may potentially serve as a convenient marker of lung
inflammation. Furthermore, the increased demand for GSH production in the
face of ongoing inflammation suggests a potential role for supplementation
with cysteine donors. (CHEST 1999; 116:201–205)
Key words: antioxidants; inflammation; oxidative
stress Abbreviations: CF 5 cystic fibrosis; GSH 5 glutathione; GSSG 5
glutathione disulfide; rpm 5 revolutions per minute; SSA 5
5-sulphosalicylic acid; TLC 5 total lung capacity; TNF-a 5 tumor necrosis
factor-a
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