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β-adrenergic sweat secretion as a diagnostic test for cystic fibrosis.
Am J Respir Crit Care Med. 2012 Oct 15; 186(8):732-9.AJ

Abstract

RATIONALE

β-Adrenergically induced sweat secretion offers an expedient method to assess native cystic fibrosis transmembrane conductance regulator (CFTR) secretory function in vivo.

OBJECTIVES

To evaluate the sensitivity, specificity, and reliability of a test based on the activity and secretory function of CFTR in the sweat gland.

METHODS

Primary and validation trials with prospectively ascertained healthy control subjects, obligate heterozygotes, and patients with a CFTR-related disorder and CF (pancreatic sufficient and insufficient).

MEASUREMENTS AND MAIN RESULTS

Diagnostic accuracy and reliability of β-adrenergic sweat secretory rates using an evaporimeter was assessed and compared with sweat chloride concentrations. The cholinergically stimulated mean sweat rate did not differ among groups. The mean maximal β-adrenergically stimulated sweat rate in heterozygotes was about half the rate of healthy control subjects, and completely absent in pancreatic-insufficient patients with CF and pancreatic-sufficient patients with CF (P < 0.0001). Subjects with a CFTR-related disorder showed reduced or absent β-adrenergic sweat secretion. The β-adrenergic secretory response demonstrated high diagnostic accuracy (area under a characteristic receiver-operator curve = 0.99; 95% confidence interval, 0.97-1.00) and reliability (intraclass correlation, 0.90; 95% confidence interval, 0.81-0.95). The diagnostic cutoff level for CF, derived from the primary trial, correctly identified all control subjects, heterozygotes, and patients with CF in the validation cohort, whereas concurrent sweat chloride measurements misclassified one heterozygote and five subjects with CF. The cholinergic and β-adrenergic sweat secretion rates were lower in women compared with men (P < 0.001).

CONCLUSIONS

β-Adrenergic sweat secretion rate determined by evaporimetry is an accurate and reliable technique to assess different levels of CFTR function and to identify patients with CF.

Authors+Show Affiliations

Department of Pediatrics, University of California-San Diego, La Jolla, CA, USA.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Comparative Study
Journal Article
Research Support, Non-U.S. Gov't
Validation Study

Language

eng

PubMed ID

22859523

Citation

Quinton, Paul, et al. "Β-adrenergic Sweat Secretion as a Diagnostic Test for Cystic Fibrosis." American Journal of Respiratory and Critical Care Medicine, vol. 186, no. 8, 2012, pp. 732-9.
Quinton P, Molyneux L, Ip W, et al. Β-adrenergic sweat secretion as a diagnostic test for cystic fibrosis. Am J Respir Crit Care Med. 2012;186(8):732-9.
Quinton, P., Molyneux, L., Ip, W., Dupuis, A., Avolio, J., Tullis, E., Conrad, D., Shamsuddin, A. K., Durie, P., & Gonska, T. (2012). Β-adrenergic sweat secretion as a diagnostic test for cystic fibrosis. American Journal of Respiratory and Critical Care Medicine, 186(8), 732-9. https://doi.org/10.1164/rccm.201205-0922OC
Quinton P, et al. Β-adrenergic Sweat Secretion as a Diagnostic Test for Cystic Fibrosis. Am J Respir Crit Care Med. 2012 Oct 15;186(8):732-9. PubMed PMID: 22859523.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - β-adrenergic sweat secretion as a diagnostic test for cystic fibrosis. AU - Quinton,Paul, AU - Molyneux,Laura, AU - Ip,Wan, AU - Dupuis,Annie, AU - Avolio,Julie, AU - Tullis,Elizabeth, AU - Conrad,Douglas, AU - Shamsuddin,A K, AU - Durie,Peter, AU - Gonska,Tanja, Y1 - 2012/08/02/ PY - 2012/8/4/entrez PY - 2012/8/4/pubmed PY - 2012/12/27/medline SP - 732 EP - 9 JF - American journal of respiratory and critical care medicine JO - Am. J. Respir. Crit. Care Med. VL - 186 IS - 8 N2 - RATIONALE: β-Adrenergically induced sweat secretion offers an expedient method to assess native cystic fibrosis transmembrane conductance regulator (CFTR) secretory function in vivo. OBJECTIVES: To evaluate the sensitivity, specificity, and reliability of a test based on the activity and secretory function of CFTR in the sweat gland. METHODS: Primary and validation trials with prospectively ascertained healthy control subjects, obligate heterozygotes, and patients with a CFTR-related disorder and CF (pancreatic sufficient and insufficient). MEASUREMENTS AND MAIN RESULTS: Diagnostic accuracy and reliability of β-adrenergic sweat secretory rates using an evaporimeter was assessed and compared with sweat chloride concentrations. The cholinergically stimulated mean sweat rate did not differ among groups. The mean maximal β-adrenergically stimulated sweat rate in heterozygotes was about half the rate of healthy control subjects, and completely absent in pancreatic-insufficient patients with CF and pancreatic-sufficient patients with CF (P < 0.0001). Subjects with a CFTR-related disorder showed reduced or absent β-adrenergic sweat secretion. The β-adrenergic secretory response demonstrated high diagnostic accuracy (area under a characteristic receiver-operator curve = 0.99; 95% confidence interval, 0.97-1.00) and reliability (intraclass correlation, 0.90; 95% confidence interval, 0.81-0.95). The diagnostic cutoff level for CF, derived from the primary trial, correctly identified all control subjects, heterozygotes, and patients with CF in the validation cohort, whereas concurrent sweat chloride measurements misclassified one heterozygote and five subjects with CF. The cholinergic and β-adrenergic sweat secretion rates were lower in women compared with men (P < 0.001). CONCLUSIONS: β-Adrenergic sweat secretion rate determined by evaporimetry is an accurate and reliable technique to assess different levels of CFTR function and to identify patients with CF. SN - 1535-4970 UR - https://www.unboundmedicine.com/medline/citation/22859523/β_adrenergic_sweat_secretion_as_a_diagnostic_test_for_cystic_fibrosis_ L2 - http://www.atsjournals.org/doi/full/10.1164/rccm.201205-0922OC?url_ver=Z39.88-2003&amp;rfr_id=ori:rid:crossref.org&amp;rfr_dat=cr_pub=pubmed DB - PRIME DP - Unbound Medicine ER -