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The Short Term Safety and Efficacy of Inhaled L-arginine in Patients With Cystic Fibrosis

Phase 2
Completed
Conditions
Cystic Fibrosis
Interventions
Registration Number
NCT00405665
Lead Sponsor
The Hospital for Sick Children
Brief Summary

The objective of this trial is to determine the safety and effect on pulmonary function of 14 days of inhaled L-arginine versus placebo administered over a period of 14 days in a cohort of CF patients.

Detailed Description

Despite the inflammatory nature of lung disease in CF, nitric oxide (NO) formation as well as the expression of NOS2 has been found to be decreased in CF airways. While the reasons for impaired airway NO formation remain incompletely understood, there is evidence that low NO formation contributes to lung pathophysiology in CF. Constitutive endogenous formation of Nitric oxide (NO) in airways is thought to play a role in neurotransmission, smooth muscle relaxation and bronchodilation. Previous animal experiments have shown that the addition of L-arginine, the precursor of enzymatic NO formation, resulted in a significantly greater relaxation of tracheas. There is also evidence that a single dose of inhaled L-arginine improves pulmonary function in CF. In this study we will assess the effect of L-arginine inhalation on lung function, nitric oxide formation, airway inflammation and bacterial infection in CF patients.

Recruitment & Eligibility

Status
COMPLETED
Sex
All
Target Recruitment
20
Inclusion Criteria
  • Diagnosis of CF as defined by two or more clinical features of CF and a documented sweat chloride concentration > 60 mEq/L and/or two well characterized disease causing CFTR gene mutations
  • 14 years of age and older at enrollment
  • Clinically stable at enrollment
  • Ability to comply with medication use, study visits and study procedures
  • FEV1 % predicted > 40% < 80 % as calculated by reference equations
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Exclusion Criteria
  • Respiratory culture positive for: B. cepacia complex within past year or at screening
  • Use of systemic corticosteroids within 30 days of screening
  • Use of intravenous antibiotics or oral quinolones within 14 days of screening
  • History of biliary cirrhosis, portal hypertension, or splenomegaly
  • Other major organ dysfunction
  • History of lung transplantation or currently on lung transplant list
  • Supplemental oxygen therapy
  • Oxygen saturation < 95 % on room air
  • Positive pregnancy test at screening
  • Investigational drug use within 30 days of screening
  • History of alcohol, illicit drug or medication abuse within 1 year of screening
  • Acute respiratory symptoms
  • Inability to take any form of bronchodilator
  • Wheezing at the time of study
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Study & Design

Study Type
INTERVENTIONAL
Study Design
CROSSOVER
Arm && Interventions
GroupInterventionDescription
2L-arginine-
1L-arginine-
Primary Outcome Measures
NameTimeMethod
Change in FEV1 (in liters) from baselineAt the end of the 14 day treatment period
Adverse events such as gastrointestinal complaints, wheezing, hepatitis or shortness of breath70 weeks
Secondary Outcome Measures
NameTimeMethod
Change in FVC and change in FEV25-75 from baseline to completion of the 2 week treatment period.Will be measured at the end of the 14 day treatment period
Change in exhaled nitric oxide (FeNO)70 days
Changes in inflammatory markers in sputum from baseline including neutrophils (sputum), neutrophil elastase (sputum) and interleukin (IL)-8 concentrations (sputum).Will me measured at the end of the 14 day treatment period
Changes in sputum concentrations of L-arginine metabolites70 days

Trial Locations

Locations (2)

The Hospital for Sick Children

🇨🇦

Toronto, Ontario, Canada

St. Michael's Hospital

🇨🇦

Toronto, Ontario, Canada

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