Low Beta-Adrenergic Sweat Responses in Cystic Fibrosis and Cystic Fibrosis Transmembrane Conductance Regulator-Related Metabolic Syndrome Children
| Autor: | Paul M. Quinton, Danieli Salinas, Colleen Azen, Lucia Kang |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2017 |
| Předmět: |
Pulmonary and Respiratory Medicine
medicine.medical_specialty Adrenergic receptor Gastroenterology Cystic fibrosis SWEAT cystic fibrosis 03 medical and health sciences 0302 clinical medicine sweat rate sweat chloride test 030225 pediatrics Internal medicine Immunology and Allergy Medicine 030212 general & internal medicine Original Research Newborn screening biology business.industry medicine.disease Cystic fibrosis transmembrane conductance regulator CRMS Endocrinology beta-adrenergic stimulation Pediatrics Perinatology and Child Health biology.protein Pilot test Metabolic syndrome Test protocol business |
| Zdroj: | Pediatric Allergy, Immunology, and Pulmonology |
| ISSN: | 2151-3228 2151-321X |
| Popis: | β-adrenergically stimulated sweat secretion depends on the function of the cystic fibrosis transmembrane conductance regulator (CFTR) and discriminates between cystic fibrosis (CF) patients and healthy controls. Therefore, we sought to determine the feasibility, safety, and efficacy of assaying β-adrenergic sweating in children identified by CF newborn screening to help determine prognoses for individuals with CFTR-related metabolic syndrome (CRMS). Preschool age children with a positive newborn screening test for CF participated in this cross-sectional study. Sweat rates were measured by evaporimetery (cyberDERM, inc.) as transepidermal water losses (g H2O/m2/h) before and after selectively stimulating sweat glands either cholinergically or β-adrenergically. Net peak sweat responses assayed as evaporation rates were compared between CF and CRMS cohorts. After a pilot test in adults, children between 4 and 6 years of age were evaluated (CF, n = 16; CRMS, n = 10). The test protocol was well tolerated; electrocardiograms and vital signs were within normal range for all subjects. The mean evaporative sweat rates in both groups in response to cholinergic stimulation were similar (CF, 60.3 ± 23.8; CRMS, 57.7 ± 13.9; p = 0.72) as well as to β-adrenergic stimulation (CF, 1.1 ± 1.7; CRMS, 2.0 ± 2.0; p = 0.14). The β-adrenergic sweat test is safe and well tolerated by young children. However, the β-adrenergic sweat secretion rates as measured by evaporimetery did not discriminate between CF and CRMS cohorts. |
| Databáze: | OpenAIRE |
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