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

Hugo R. De Jonge; Manfred Ballmann; Henk Veeze; Inez Bronsveld; Frauke Stanke; Burkhard Tümmler; Maarten Sinaasappel

doi:10.1016/j.jcf.2004.05.034

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

Hugo R. De Jonge^*, Manfred Ballmann, Henk Veeze, Inez Bronsveld, Frauke Stanke, Burkhard Tümmler, Maarten Sinaasappel

^*Corresponding author for this work

Research output: Contribution to journal › Review article › peer-review

57 Citations (Scopus)

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.

Original language	English
Pages (from-to)	159-163
Number of pages	5
Journal	Journal of Cystic Fibrosis
Volume	3
Issue number	SUPPL. 2
DOIs	https://doi.org/10.1016/j.jcf.2004.05.034
Publication status	Published - Aug 2004
Externally published	Yes

Keywords

Chlorides
Cystic Fibrosis
Cystic Fibrosis Transmembrane Conductance Regulator
Humans
Intestinal Mucosa
Ion Transport
Patch-Clamp Techniques
Rectum
Journal Article
Research Support, Non-U.S. Gov't
Review

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10.1016/j.jcf.2004.05.034

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

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.",

keywords = "Chlorides, Cystic Fibrosis, Cystic Fibrosis Transmembrane Conductance Regulator, Humans, Intestinal Mucosa, Ion Transport, Patch-Clamp Techniques, Rectum, Journal Article, Research Support, Non-U.S. Gov't, Review",

author = "{De Jonge}, {Hugo R.} and Manfred Ballmann and Henk Veeze and Inez Bronsveld and Frauke Stanke and Burkhard T{\"u}mmler and Maarten Sinaasappel",

year = "2004",

month = aug,

doi = "10.1016/j.jcf.2004.05.034",

language = "English",

volume = "3",

pages = "159--163",

journal = "Journal of Cystic Fibrosis",

issn = "1569-1993",

publisher = "Elsevier",

number = "SUPPL. 2",

}

TY - JOUR

T1 - Ex vivo CF diagnosis by intestinal current measurements (ICM) in small aperture, circulating Ussing chambers

AU - De Jonge, Hugo R.

AU - Ballmann, Manfred

AU - Veeze, Henk

AU - Bronsveld, Inez

AU - Stanke, Frauke

AU - Tümmler, Burkhard

AU - Sinaasappel, Maarten

PY - 2004/8

Y1 - 2004/8

N2 - 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.

AB - 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.

KW - Chlorides

KW - Cystic Fibrosis

KW - Cystic Fibrosis Transmembrane Conductance Regulator

KW - Humans

KW - Intestinal Mucosa

KW - Ion Transport

KW - Patch-Clamp Techniques

KW - Rectum

KW - Journal Article

KW - Research Support, Non-U.S. Gov't

KW - Review

UR - http://www.scopus.com/inward/record.url?scp=11044232030&partnerID=8YFLogxK

U2 - 10.1016/j.jcf.2004.05.034

DO - 10.1016/j.jcf.2004.05.034

M3 - Review article

C2 - 15463951

SN - 1569-1993

VL - 3

SP - 159

EP - 163

JO - Journal of Cystic Fibrosis

JF - Journal of Cystic Fibrosis

IS - SUPPL. 2

ER -

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

Abstract

Keywords

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