Colonic and esophageal transepithelial potential difference in cystic fibrosis

Roy C. Orlando, Don W. Powell, Robert D. Croom, Helen M. Berschneider, Richard C. Boucher, Michael R. Knowles

Research output: Contribution to journalArticle

28 Citations (Scopus)

Abstract

To evaluate differences in the expression of cystic fibrosis (CF) transport defects in the gastrointestinal tract of subjects with CF, in vivo measurements of colonic and esophageal transepithelial electrical potential difference (PD) were performed before and during amiloride superfusion in CF and healthy subjects. Esophageal PD before (-16 ± 2 vs. -16 ± 3 mV) and after (-14 ± 2 vs. -15 ± 0.3 mV) superfusion with amiloride were similar for CF and healthy subjects. Basal rectosigmoid colon PD was also similar (CF: mean -23 ± 6 and maximal -37 ± 9 mV; normal: mean -26 ± 5 and maximal -45 ± 11 mV) in both groups. However, with amiloride superfusion (10-4 M) the colonic PD in CF subjects was almost abolished (95% ± 15% inhibition), whereas the PD in healthy subjects was only partially reduced (42% ± 6%) (p < 0.05). The greater inhibition with amiloride in CF, which was evident in absolute terms (26 ± 4 vs. 16 ± 3 mV for controls, p < 0.05) as well as relative terms, could not be ascribed to a difference in mineralocorticoid secretion rates, because 24-h urine excretion of aldosterone and 17 hydroxy and 17 ketosteroids were similar in both groups. Freshly excised colonic epithelia from 1 CF and 3 non-CF subjects were studied in Ussing chambers, and a similar difference in amiloride responsiveness noted: PD and short-circuit current declined 33% ± 2% and 37% ± 4%, respectively, in seven tissues from the colons of 3 patients without CF, whereas both PD and short-circuit current were fully inhibited (100%) in all three tissues from the CF patient. As the presence of an amiloride-insensitive component of short-circuit current in non-CF colon is largely due to electrogenic Cl- secretion, the demonstration that this component was absent both in vivo and in vitro in CF colon establishes the presence of a defect in electrolyte transport in CF colon, a defect consistent with recent reports of absent electrogenic Cl- secretion in CF intestine.

Original languageEnglish
Pages (from-to)1041-1048
Number of pages8
JournalGastroenterology
Volume96
Issue number4
StatePublished - 1989
Externally publishedYes

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Cystic Fibrosis
Amiloride
Colon
Healthy Volunteers
Fibrosis
17-Ketosteroids
Mineralocorticoids
Aldosterone
Electrolytes
Intestines
Gastrointestinal Tract
Epithelium
Urine

ASJC Scopus subject areas

  • Gastroenterology

Cite this

Orlando, R. C., Powell, D. W., Croom, R. D., Berschneider, H. M., Boucher, R. C., & Knowles, M. R. (1989). Colonic and esophageal transepithelial potential difference in cystic fibrosis. Gastroenterology, 96(4), 1041-1048.

Colonic and esophageal transepithelial potential difference in cystic fibrosis. / Orlando, Roy C.; Powell, Don W.; Croom, Robert D.; Berschneider, Helen M.; Boucher, Richard C.; Knowles, Michael R.

In: Gastroenterology, Vol. 96, No. 4, 1989, p. 1041-1048.

Research output: Contribution to journalArticle

Orlando, RC, Powell, DW, Croom, RD, Berschneider, HM, Boucher, RC & Knowles, MR 1989, 'Colonic and esophageal transepithelial potential difference in cystic fibrosis', Gastroenterology, vol. 96, no. 4, pp. 1041-1048.
Orlando RC, Powell DW, Croom RD, Berschneider HM, Boucher RC, Knowles MR. Colonic and esophageal transepithelial potential difference in cystic fibrosis. Gastroenterology. 1989;96(4):1041-1048.
Orlando, Roy C. ; Powell, Don W. ; Croom, Robert D. ; Berschneider, Helen M. ; Boucher, Richard C. ; Knowles, Michael R. / Colonic and esophageal transepithelial potential difference in cystic fibrosis. In: Gastroenterology. 1989 ; Vol. 96, No. 4. pp. 1041-1048.
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abstract = "To evaluate differences in the expression of cystic fibrosis (CF) transport defects in the gastrointestinal tract of subjects with CF, in vivo measurements of colonic and esophageal transepithelial electrical potential difference (PD) were performed before and during amiloride superfusion in CF and healthy subjects. Esophageal PD before (-16 ± 2 vs. -16 ± 3 mV) and after (-14 ± 2 vs. -15 ± 0.3 mV) superfusion with amiloride were similar for CF and healthy subjects. Basal rectosigmoid colon PD was also similar (CF: mean -23 ± 6 and maximal -37 ± 9 mV; normal: mean -26 ± 5 and maximal -45 ± 11 mV) in both groups. However, with amiloride superfusion (10-4 M) the colonic PD in CF subjects was almost abolished (95{\%} ± 15{\%} inhibition), whereas the PD in healthy subjects was only partially reduced (42{\%} ± 6{\%}) (p < 0.05). The greater inhibition with amiloride in CF, which was evident in absolute terms (26 ± 4 vs. 16 ± 3 mV for controls, p < 0.05) as well as relative terms, could not be ascribed to a difference in mineralocorticoid secretion rates, because 24-h urine excretion of aldosterone and 17 hydroxy and 17 ketosteroids were similar in both groups. Freshly excised colonic epithelia from 1 CF and 3 non-CF subjects were studied in Ussing chambers, and a similar difference in amiloride responsiveness noted: PD and short-circuit current declined 33{\%} ± 2{\%} and 37{\%} ± 4{\%}, respectively, in seven tissues from the colons of 3 patients without CF, whereas both PD and short-circuit current were fully inhibited (100{\%}) in all three tissues from the CF patient. As the presence of an amiloride-insensitive component of short-circuit current in non-CF colon is largely due to electrogenic Cl- secretion, the demonstration that this component was absent both in vivo and in vitro in CF colon establishes the presence of a defect in electrolyte transport in CF colon, a defect consistent with recent reports of absent electrogenic Cl- secretion in CF intestine.",
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N2 - To evaluate differences in the expression of cystic fibrosis (CF) transport defects in the gastrointestinal tract of subjects with CF, in vivo measurements of colonic and esophageal transepithelial electrical potential difference (PD) were performed before and during amiloride superfusion in CF and healthy subjects. Esophageal PD before (-16 ± 2 vs. -16 ± 3 mV) and after (-14 ± 2 vs. -15 ± 0.3 mV) superfusion with amiloride were similar for CF and healthy subjects. Basal rectosigmoid colon PD was also similar (CF: mean -23 ± 6 and maximal -37 ± 9 mV; normal: mean -26 ± 5 and maximal -45 ± 11 mV) in both groups. However, with amiloride superfusion (10-4 M) the colonic PD in CF subjects was almost abolished (95% ± 15% inhibition), whereas the PD in healthy subjects was only partially reduced (42% ± 6%) (p < 0.05). The greater inhibition with amiloride in CF, which was evident in absolute terms (26 ± 4 vs. 16 ± 3 mV for controls, p < 0.05) as well as relative terms, could not be ascribed to a difference in mineralocorticoid secretion rates, because 24-h urine excretion of aldosterone and 17 hydroxy and 17 ketosteroids were similar in both groups. Freshly excised colonic epithelia from 1 CF and 3 non-CF subjects were studied in Ussing chambers, and a similar difference in amiloride responsiveness noted: PD and short-circuit current declined 33% ± 2% and 37% ± 4%, respectively, in seven tissues from the colons of 3 patients without CF, whereas both PD and short-circuit current were fully inhibited (100%) in all three tissues from the CF patient. As the presence of an amiloride-insensitive component of short-circuit current in non-CF colon is largely due to electrogenic Cl- secretion, the demonstration that this component was absent both in vivo and in vitro in CF colon establishes the presence of a defect in electrolyte transport in CF colon, a defect consistent with recent reports of absent electrogenic Cl- secretion in CF intestine.

AB - To evaluate differences in the expression of cystic fibrosis (CF) transport defects in the gastrointestinal tract of subjects with CF, in vivo measurements of colonic and esophageal transepithelial electrical potential difference (PD) were performed before and during amiloride superfusion in CF and healthy subjects. Esophageal PD before (-16 ± 2 vs. -16 ± 3 mV) and after (-14 ± 2 vs. -15 ± 0.3 mV) superfusion with amiloride were similar for CF and healthy subjects. Basal rectosigmoid colon PD was also similar (CF: mean -23 ± 6 and maximal -37 ± 9 mV; normal: mean -26 ± 5 and maximal -45 ± 11 mV) in both groups. However, with amiloride superfusion (10-4 M) the colonic PD in CF subjects was almost abolished (95% ± 15% inhibition), whereas the PD in healthy subjects was only partially reduced (42% ± 6%) (p < 0.05). The greater inhibition with amiloride in CF, which was evident in absolute terms (26 ± 4 vs. 16 ± 3 mV for controls, p < 0.05) as well as relative terms, could not be ascribed to a difference in mineralocorticoid secretion rates, because 24-h urine excretion of aldosterone and 17 hydroxy and 17 ketosteroids were similar in both groups. Freshly excised colonic epithelia from 1 CF and 3 non-CF subjects were studied in Ussing chambers, and a similar difference in amiloride responsiveness noted: PD and short-circuit current declined 33% ± 2% and 37% ± 4%, respectively, in seven tissues from the colons of 3 patients without CF, whereas both PD and short-circuit current were fully inhibited (100%) in all three tissues from the CF patient. As the presence of an amiloride-insensitive component of short-circuit current in non-CF colon is largely due to electrogenic Cl- secretion, the demonstration that this component was absent both in vivo and in vitro in CF colon establishes the presence of a defect in electrolyte transport in CF colon, a defect consistent with recent reports of absent electrogenic Cl- secretion in CF intestine.

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