Ex vivo CF diagnosis by intestinal current measurements (ICM) in small aperture, circulating Ussing chambers.
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.
De Jonge
Hugo R
HR
Departments of Paediatric Gastroenterology and Biochemistry, Erasmus University Medical Centre, P.O. Box 1738, Rotterdam, 3000DR The Netherlands. h.dejonge@erasmusmc.nl
Ballmann
Manfred
M
Veeze
Henk
H
Bronsveld
Inez
I
Stanke
Frauke
F
Tümmler
Burkhard
B
Sinaasappel
Maarten
M
D002712:Chlorides / Q000378:metabolism; D003550:Cystic Fibrosis / Q000175:diagnosis* / Q000378:metabolism / Q000503:physiopathology*; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000378:metabolism; D006801:Humans; D007413:Intestinal Mucosa / Q000378:metabolism*; D017136:Ion Transport / Q000502:physiology; D018408:Patch-Clamp Techniques / Q000295:instrumentation*; D012007:Rectum / Q000378:metabolism
C505032:CFTR protein, human; D002712:Chlorides; D019005:Cystic Fibrosis Transmembrane Conductance Regulator