Cystic Fibrosis Diagnosis in Newborns, Children, and Adults

Carlo Castellani, MD; Barry Linnane, MB, BCh, BAO, DCH, MRCPI, MRCPCH, MD; Iwona Pranke, PhD; Federico Cresta, MD; Isabelle Sermet-Gaudelus, MD, PhD; Daniel Peckham, MD

Disclosures

Semin Respir Crit Care Med. 2019;40(6):701-714. 

In This Article

Evaporimetry

Sweat secretion can follow either cholinergic or β-adrenergic pathways.[153] The former, which is essential for thermoregulation, is stimulated during the sweat test and is not affected in CF, as the sweat test only reflects the defect in ion reabsorption by sweat tubules. The latter, whose physiologic purpose is poorly understood, is CFTR-dependent and therefore provides a unique evaluation of the purely secretory function of CFTR in vivo. Assessment of water loss with a sensitive evaporimeter enables to assess these two pathways[154] and seems to be able to tell apart different levels of CFTR activity within the spectrum of CF disease.[154,155] Further studies are needed to gain more knowledge on the relevance of this test.

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