CFDB - Cystic Fibrosis DataBase

primary studies published RCT

Temporal associations among energy intake, plasma linoleic acid, and growth improvement in response to treatment initiation after diagnosis of cystic fibrosis.

Study design (if review, criteria of inclusion for studies)

single-centre, randomised, double-blinded, placebo-controlled phase II clinical study to test safety and efficacy

Participants

21 patients (DeltaF508 homo/heterozygous, FEV1>40% pred.) were included in the study

Interventions

12-week therapy with low-dose (700 mg/daily) or high-dose (2800 mg/daily) of NAC. After a 3-weeks placebo run-in phase, 11 patients received low-dose NAC, and 10 patients received high-dose NAC.

Outcome measures

Outcomes included safety and clinical parameters, inflammatory (total leukocyte numbers, cell differentials, TNF-alpha, IL-8) measures in induced sputum, and concentrations of extracellular glutathione in induced sputum and blood

Main results

High-dose NAC was a well-tolerated and safe medication. High-dose NAC did not alter clinical or inflammatory parameters. However, extracellular glutathione in induced sputum tended to increase on high-dose NAC.

Authors' conclusions

High-dose NAC is a well-tolerated and safe medication for a prolonged therapy of patients with CF with a potential to increase extracellular glutathione in CF airways.

Keywords: Acetylcysteine; Adult; Antioxidants; Airway clearance drugs -expectorants- mucolytic- mucociliary-; Glutathione; High-Dose; N Acetylcysteine; pharmacological_intervention; thiols; Low-Dose; Respiratory System Agents; Nacystelyn;