The Journal of pediatrics
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The Journal of pediatrics · Dec 2000
A simplified cyclic adenosine monophosphate-mediated sweat rate test for quantitative measure of cystic fibrosis transmembrane regulator (CFTR) function.
Sweat production is stimulated by both cholinergic and beta-adrenergic pathways in the sweat gland secretory coil. beta-Adrenergic pathway-mediated sweating is absent in cystic fibrosis (CF) because cyclic adenosine monophosphate (cAMP)-mediated chloride transport through the cystic fibrosis transmembrane regulator (CFTR) is disrupted. We report the development of a rapid, reproducible, macroscopic, and quantitative methodology to test the hypothesis that beta-adrenergic sweat rate discriminates among 3 different CFTR phenotypes-CF, heterozygote CF carriers, and non-CF. ⋯ Median cAMP-mediated sweat rates were 1.45 mg/20 min (CF, n = 29), 2.55 mg/20 min (CF heterozygote carriers, n = 30), and 3.65 mg/20 min (non-CF, n = 30) and were significantly different in all 3 groups (P =.0001, Kruskal-Wallis test). Methacholine-stimulated sweat rates were similar for all 3 groups. The cAMP-mediated sweat rate test may be a useful endpoint for studies of new agents to increase the function of CFTR.
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The Journal of pediatrics · Dec 2000
Randomized Controlled Trial Clinical TrialThe Preschool Respiratory Assessment Measure (PRAM): a responsive index of acute asthma severity.
To elaborate and validate a Preschool Respiratory Assessment Measure (PRAM) that would accurately reflect the severity of airway obstruction and the response to treatment in young patients with asthma. ⋯ PRAM appears to be a responsive but moderately discriminative tool for assessing acute asthma severity. This measure, designed for preschool-aged children, has been validated against a concurrent measure of lung function.