These data suggest that a single inhalation of l-arginine acutely and transiently improves pulmonary function in CF through the formation of nitric oxide. Augmentation of airway nitric oxide formation by inhalation of l-arginine is a promising therapeutic approach in patients with CF.
Exhaled nitric oxide (eNO) is decreased in cystic fibrosis (CF). The effect of oral Larginine, the precursor of enzymatic nitric oxide (NO) formation, on airway NO in patients with CF was studied.In a pilot study, oral L-arginine was given in a single dose of 200 mg?kg -1 body weight to eight healthy controls and eight CF patients. Subsequently, the same L-arginine dose was given to 10 patients with CF (five females) t.i.d. for 6 weeks in a randomised double-blind placebo-controlled crossover study.A single dose of oral L-arginine resulted in a 5.5-fold increase of L-arginine in plasma and a 1.3-fold increase of L-arginine in sputum after 2 h. Maximum eNO, within 3 h of L-arginine intake, increased significantly in both CF patients (5.4¡2.1 ppb versus 8.3¡3.5 ppb) and controls (18.0¡8.1 ppb versus 26.4¡12.3 ppb). Supplementation of L-arginine for 6 weeks resulted in a sustained increase in eNO compared to placebo (9.7¡5.7 ppb versus 6.3¡3.1 ppb). An effect of L-arginine supplementation on forced expiratory volume in one second was not observed.These data indicate that airway nitric oxide formation in cystic fibrosis patients can be augmented with oral L-arginine supplementation.
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