Failure to induce secretion in jejunal biopsies from children with cystic fibrosis.

Taylor, Christopher J.; Baxter, P S; Hardcastle, J; Hardcastle, P T

doi:10.1136/gut.29.7.957

Cited by 132 publications

(73 citation statements)

References 18 publications

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“…These observations were supported by the subsequent studies showing increased nutrientdependent short circuit currents (Baxter et al , 1989(Baxter et al , 1990aHardcastle et al 1990;Teune et al 1996). It is well known that secretion of electrolyte fluid is severely compromised throughout the intestine including the jejunum and ileum (Berschneider et al 1987(Berschneider et al , 1988Taylor et al 1987Taylor et al , 1988. Because the nutrient absorption is driven by the driving force for Na þ , absence of a Cl 2 shunt due to lack of CFTR anion channels in the plasma membranes of CF intestinal epithelium seems to increase the driving force for Na þ , causing a significant surge in glucose and other nutrient absorption.…”

Section: Small Intestinesupporting

confidence: 73%

Status of Fluid and Electrolyte Absorption in Cystic Fibrosis

Reddy

Stutts

2013

Cold Spring Harbor Perspectives in Medicine

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Section: Small Intestinesupporting

confidence: 73%

Status of Fluid and Electrolyte Absorption in Cystic Fibrosis

Reddy

Stutts

2013

Cold Spring Harbor Perspectives in Medicine

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“…No studies were found on reliability. ICM has been shown to discriminate between patients with cystic fibrosis and healthy individuals [37,[80][81][82][83][84][85][86]] and, at a group level, can discriminate between pancreatic sufficient and insufficient patients [35]. Similar to the sweat test and the NPD, patients with cystic fibrosis who were grouped according to their ICM result have been shown to differ in disease presentation: the more chloride secretion measured in the rectal mucosa, the milder the disease presentation [35,37,82].…”

Section: Clinimetrics Of Cftr Bio-assaysmentioning

confidence: 96%

CFTR biomarkers: time for promotion to surrogate end-point

et al. 2012

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In patients with cystic fibrosis, cystic fibrosis transmembrane conductance regulator (CFTR) biomarkers, such as sweat chloride concentration and/or nasal potential difference, are used as end-points of efficacy in phase-III clinical trials with the disease modifying drugs ivacaftor (VX-770), VX809 and ataluren. The aim of this project was to review the literature on reliability, validity and responsiveness of nasal potential difference, sweat chloride and intestinal current measurement in patients with cystic fibrosis.Data on clinimetric properties were collected for each biomarker and reviewed by an international team of experts. Data on reliability, validity and responsiveness were tabulated. In addition, narrative answers to four key questions were discussed and agreed by the team of experts.The data collected demonstrated the reliability, validity and responsiveness of nasal potential difference. Fewer data were found on reliability of sweat chloride concentration; however, validity and responsiveness were demonstrated. Validity was demonstrated for intestinal current measurement, but further information is required on reliability and responsiveness. For all three end-points, normal values were collected and further research requirements were proposed.This body of work adds useful information to support the promotion of CFTR biomarkers to surrogate end-points and to guide further research in the area.

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“…The electrical activity of biopsy specimens was measured as described previously (3). Each tissue was mounted as a sheet (exposed area, 3 mm2) in a mini-Ussing chamber and incubated at 37°C with Krebs bicarbonate saline gassed with 95% 0215% CO2.…”

Section: Methodsmentioning

confidence: 99%