Ex vivo CF diagnosis by intestinal current measurements (ICM) in small aperture, circulating Ussing chambers

J Cyst Fibros. 2004 Aug:3 Suppl 2:159-63. doi: 10.1016/j.jcf.2004.05.034.

Abstract

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.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Chlorides / metabolism
  • Cystic Fibrosis / diagnosis*
  • Cystic Fibrosis / metabolism
  • Cystic Fibrosis / physiopathology*
  • Cystic Fibrosis Transmembrane Conductance Regulator / metabolism
  • Humans
  • Intestinal Mucosa / metabolism*
  • Ion Transport / physiology
  • Patch-Clamp Techniques / instrumentation*
  • Rectum / metabolism

Substances

  • CFTR protein, human
  • Chlorides
  • Cystic Fibrosis Transmembrane Conductance Regulator