Glutathione

Question

Hello
Could you please give us information regarding reduced Glutathione? Is there any deficiency for CF patients (that would be expected) ? Is this deficiency associated with some symptoms in particular? Is there any research done with external supply of Glutathione? What type of Glutathione? What are the results?

Answer

Hello,
Oxidative stress is a biological response that plays an important role in some diseases (as infections, inflammatory diseases, cardiovascular diseases)…and this is the situation in the lung, notably in CF where neutrophils recruited by the presence of pathogens release oxidative substances. Glutathione (GSH) is an antioxidant existing in airways which may, according to some research, be transported by CFTR through cellular membranes, this may explain the lower quantity of GSH found from infancy on in CF patients’ airways. In lungs, GSH could act by reducing the effect of the toxin of Pseudomonas aeruginosa with whom it is combined.
Several trials to provide GSH by inhalation or oral way have been performed but a Cochrane review published in 2013 showed a lack of efficacy whatever the administration way used was: 9 clinical trials, 225 patients (6 trials concerning the oral way and 3 the inhalation). This review also showed a lack of high quality trials and the necessity to continue investigations on the topic.
In 2015 a randomized double-blind, placebo-controlled study to evaluate the efficacy of a 6 months treatment of inhaled GSH in 44 children with CF under 10 years (Visca et al. J Pediatr Gastroenterol Nutr) showed a significant improvement of nutritional status and growth (heigh, weight; BMI) and also a reduction of inflammation, hepatic inflammation markers and intestinal inflammation markers (fecal calprotectin), suggesting a GSH efficacy in the fight against intestinal inflammation et its systemic consequences.
In 2015, too, a 12 months randomized study in subjects with CF (54 adults and 51 children) receiving inhaled GSH, showed a significant improvement of FEV1 and of the 6 min walk-test, only in subjects with a non-severe disease.

In conclusion, scientist proofs based on high quality trials are needed, but some data already suggest significane of GSH in CF treatment (pulmonal, hepatic, intestinal).

Best regards
Virginie Colomb-Jung, M.D
Head of medical department
Vaincre la mucoviscidose

06.06.2016