A multicenter evaluation of sweat chloride concentration and variation in infants with cystic fibrosis.

Fifty-nineCF infants' sweat chloride concentrations were analyzed to answer the questions: What is the biological and analytical variation in sweat chloride concentrations collected from the 32 infants homozygous for the F508 deletion? Do sweat chloride concentrations change in the first year of life beyond the variance previously established for adults with similar CFTR mutations? The biological and analytical variation of the infants' sweat chloride concentration was similar to that seen in adult CF patients. While there was a statistically significant difference between sweat chloride concentration in early (89.8 mmol/L) and late (95.

Novel Application of Aptamer Proteomic Analysis in Cystic Fibrosis Bronchoalveolar Lavage Fluid.

Purpose: Biomarkers are needed in cystic fibrosis (CF) to understand disease progression, assess response to therapy, and enrich enrollment for clinical trials. Aptamer-based proteomics have proven useful in blood samples. The aim is to evaluate proteins in bronchoalveolar lavage fluid (BALF) in CF children compared to controls and identify endotypes during CF exacerbations.

Characteristics and outcomes of oral antibiotic treated pulmonary exacerbations in children with cystic fibrosis.

Background: Pulmonary exacerbations (PEx) in children with cystic fibrosis (CF) are frequently treated in the outpatient setting with oral antibiotics. However, little is known about the characteristics of PEx managed on an outpatient basis and the effectiveness of oral antibiotic therapy. We sought to prospectively evaluate clinical and laboratory changes associated with oral antibiotic treatment for PEx.

Azithromycin for Early Pseudomonas Infection in Cystic Fibrosis. The OPTIMIZE Randomized Trial.

Rationale: New isolation of Pseudomonas aeruginosa (Pa) is generally treated with inhaled antipseudomonal antibiotics such as tobramycin inhalation solution (TIS). A therapeutic approach that complements traditional antimicrobial therapy by reducing the risk of pulmonary exacerbation and inflammation may ultimately prolong the time to Pa recurrence.

Objectives: To test the hypothesis that the addition of azithromycin to TIS in children with cystic fibrosis and early Pa decreases the risk of pulmonary exacerbation and prolongs the time to Pa recurrence.