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.
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The Journal of pediatrics · Nov 2000
Neurodevelopmental status of newborns and infants with congenital heart defects before and after open heart surgery.
Neurodevelopmental disabilities in children with congenital heart defects (CHDs) have been primarily attributed to intraoperative events without consideration of preoperative and postoperative factors. ⋯ Neurodevelopmental abnormalities are common in young infants with CHDs and are often present before open heart surgery. These developmental concerns are clinically underappreciated. Early systematic developmental screening may be warranted in this population of interest.
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To evaluate the morbidity and mortality of preterm infants with congenital heart disease (CHD), a chart review was performed for infants with CHD, excluding isolated patent ductus arteriosus, who were <37 weeks' gestation, weighed <2500 g, and were admitted to our neonatal intensive care unit from 1976 to 1999 (N = 201). ⋯ Infants with both CHD and prematurity did significantly worse than either group alone. Such outcome data are required for proper allocation of resources to care for this high-risk pediatric population.
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The Journal of pediatrics · Nov 2000
Treatment choices for extremely preterm infants: an international perspective.
To compare treatment choices of neonatal physicians and nurses in 11 European countries for a hypothetical case of extreme prematurity (24 weeks' gestational age, birth weight of 560 g, Apgar score of 1 at 1 minute). ⋯ An extremely premature infant is regarded as viable by most physicians, whereas after deterioration of the clinical conditions decision-making patterns vary according to country. These findings have implications for the ethical debate surrounding treatment of infants of borderline viability and for the interpretation and comparison of international statistics.