Intestinal current measurements (ICM) on rectal suction biopsies are a tool for the ex vivo diagnosis of classical and atypical cystic fibrosis (CF). We present the basic ICM protocol, typical tracings and their interpretation. The ICM technique allows the registration of CF-induced changes in electrogenic transepithelial ion transport (Cl-, HCO3-, K+) in a Cl- secretory epithelium, and on the basis of pharmacological criteria, is able to discriminate between CFTR-mediated Cl- secretion and secretion through alternative anion channels. ICM is particularly useful for the classification of individuals with CF-like clinical features with equivocal sweat test values and/or no or one identifiable CFTR mutation.

CFTR, Classification of cystic fibrosis, Epithelial chloride secretion, Intestinal epithelium, Ussing chamber
dx.doi.org/10.1016/j.jcf.2004.05.034, hdl.handle.net/1765/68085
Journal of Cystic Fibrosis
Department of Pediatrics

de Jonge, H.R, Ballmann, M, Veeze, H.J, Bronsveld, I, Stanke, F, Tümmler, B, & Sinaasappel, M. (2004). Ex vivo CF diagnosis by intestinal current measurements (ICM) in small aperture, circulating Ussing chambers. Journal of Cystic Fibrosis, 3(SUPPL. 2), 159–163. doi:10.1016/j.jcf.2004.05.034