Local and Systemic Alterations of the L-Arginine/Nitric Oxide Pathway in Sputum, Blood, and Urine of Pediatric Cystic Fibrosis Patients and Effects of Antibiotic Treatment.

摘要: Alterations in the L-arginine (Arg)/nitric oxide (NO) pathway have been reported cystic fibrosis (CF; OMIM 219700) as result of various factors including systemic and local inflammatory activity airways. The aim present study was to evaluate Arg/NO metabolism pediatric CF patients with special emphasis on lung impairment antibiotic treatment. Seventy 78 healthy controls were included study. (43% male, median age 11.8 years) showed moderately impaired functions (FEV1 90.5 ± 19.1% (mean SD); 21 (30%) had a chronic Pseudomonas aeruginosa (PSA) infection, 24 (33%) an acute exacerbation). Plasma, urinary, sputum concentrations main metabolites, nitrate, nitrite, Arg, homoarginine (hArg), asymmetric dimethylarginine (ADMA) determined age-matched controls. Clinical parameters function infection PSA. Additionally, samples five analyzed before after routine therapy. low fractionally exhaled NO (FENO) lower plasma Arg nitrate concentrations. During exacerbation, hArg levels high dropped treatment: Arg: pre-antibiotics: 4.14 nmol/25 mg vs. post-antibiotics: 2.33 sputum, p = 0.008; hArg: 0.042 0.029 0.035. activated stable may be inflammation. PSA did not play major role regarding these differences. Exacerbation increased therapy decreased