scholarly journals Low Beta-Adrenergic Sweat Responses in Cystic Fibrosis and Cystic Fibrosis Transmembrane Conductance Regulator-Related Metabolic Syndrome Children

2017 ◽  
Vol 30 (1) ◽  
pp. 2-6 ◽  
Author(s):  
Danieli Barino Salinas ◽  
Lucia Kang ◽  
Colleen Azen ◽  
Paul Quinton
Keyword(s):  
Metabolic Syndrome ◽  
Cystic Fibrosis ◽  
Transmembrane Conductance Regulator ◽  
Transmembrane Conductance ◽  
Beta Adrenergic ◽  
Cystic Fibrosis Transmembrane

scholarly journals International Approaches to Management of CFTR-Related Metabolic Syndrome/Cystic Fibrosis Screen Positive, Inconclusive Diagnosis

2022 ◽  
Vol 8 (1) ◽  
pp. 5
Author(s):  
Jane Chudleigh ◽  
Jürg Barben ◽  
Clement L. Ren ◽  
Kevin W. Southern
Keyword(s):  
Metabolic Syndrome ◽  
Cystic Fibrosis ◽  
Health Professionals ◽  
Online Survey ◽  
Transmembrane Conductance Regulator ◽  
Transmembrane Conductance ◽  
Online Questionnaire ◽  
The Us ◽  
The Uk ◽  
Cystic Fibrosis Transmembrane

The main aim of the present study was to explore health professionals’ reported experiences and approaches to managing children who receive a designation of cystic fibrosis transmembrane conductance regulator-related metabolic syndrome/cystic fibrosis screen positive inconclusive diagnosis following a positive NBS result for cystic fibrosis. An online questionnaire was distributed via Qualtrics Survey Software and circulated to a purposive, international sample of health professionals involved in managing children with this designation. In total, 101 clinicians completed the online survey: 39 from the US, six from Canada, and 56 from Europe (including the UK). Results indicated that while respondents reported minor deviations in practice, they were cognizant of recommendations in the updated guidance and for the most part, attempted to implement these into practice consistently internationally. Where variation was reported, the purpose of this appeared to be to enable clinicians to respond to either clinical assessments or parental anxiety in order to improve outcomes for the child and family. Further research is needed to determine if these findings are reflective of both a wider audience of clinicians and actual (rather than reported) practice.

Distinct cellular mechanisms of cholinergic and beta-adrenergic sweat secretion

1996 ◽  
Vol 271 (2) ◽  
pp. C486-C494 ◽  
Author(s):  
M. M. Reddy ◽  
C. L. Bell
Keyword(s):  
Cystic Fibrosis ◽  
Sweat Glands ◽  
Transmembrane Conductance Regulator ◽  
Transmembrane Conductance ◽  
Cellular Mechanisms ◽  
Beta Adrenergic ◽  
Cholinergic Agonist ◽  
Sweat Secretion ◽  
Cystic Fibrosis Transmembrane ◽  
Beta Adrenergic Agonist

The cholinergic and beta-adrenergic sweat secretions from human sweat glands differ with respect to secretory rates and their susceptibility to cystic fibrosis (CF). Using the cultured beta-adrenergic-sensitive sweat secretory cell, we sought to determine the intracellular electrophysiological mechanisms underlying these functional differences. We found that the cholinergic agonist methacholine (10(-6) M) induced a Ca(2+)-dependent biphasic membrane potential (Vm) response: an initial hyperpolarization and a secondary depolarization. The initial hyperpolarization was independent of bath Cl- and dependent on transmembrane K+ gradient. However, the secondary depolarization of Vm was dependent on bath Cl-. In contrast, the beta-adrenergic agonist isoproterenol (10(-5) M) induced a monophasic depolarization of Vm. This depolarization was 1) dependent on bath Cl-, 2) independent of K+ conductance (GK) blocker Ba2+ (5mM), 3) unaffected by the methacholine-induced secondary depolarization of Vm, and 4) absent in cells derived from CF subjects. These results indicated that the cholinergic agonist-induced secretion mainly involves the activation of Ca(2+)-dependent GK and Cl- conductance (GCl), whereas the beta-adrenergic secretion seems to mainly depend on the activation of cystic fibrosis transmembrane conductance regulator-GCl.

scholarly journals International Approaches to Management of CFTR-Related Metabolic Syndrome / Cystic Fibrosis Screen Positive, Inconclusive Diagnosis

2021 ◽  
Author(s):  
Jane Chudleigh ◽  
Jürg Barben ◽  
Clement L Ren ◽  
Kevin W Southern
Keyword(s):  
Metabolic Syndrome ◽  
Cystic Fibrosis ◽  
Health Professionals ◽  
Online Survey ◽  
Transmembrane Conductance Regulator ◽  
Transmembrane Conductance ◽  
Online Questionnaire ◽  
The Us ◽  
The Uk ◽  
Cystic Fibrosis Transmembrane

The main aim of the present study was to explore health professionals’ reported experiences and approaches to managing children who receive a designation of cystic fibrosis transmembrane conductance regulator-related metabolic syndrome/cystic fibrosis screen positive inconclusive diagnosis following a positive NBS result for cystic fibrosis. An online questionnaire was distributed via Qualtrics Survey Software and circulated to a purposive, international sample of health professionals involved in managing children with this designation. In total, 101 clinicians completed the online survey; 39 from the US, six from Canada and 56 from Europe (including the UK). Results indicated that while respondents reported minor deviations in practice, they were cognizant of recommendations in the updated guidance and for the most part, attempted to implement these into practice consistently internationally. Where variation was reported, the purpose of this appeared to be to enable clinicians to respond to either clinical assessments or parental anxiety in order to improve outcomes for the child and family. Further research is needed to determine if these findings are reflective of both a wider audience of clinicians and actual (rather than reported) practice.

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