Assessment and pharmacological correction of abnormalities in chloride (Cl-), bicarbonate (HCO3-) and mucus transport in rectal biopsies of CF patients

Assessment and pharmacological correction of abnormalities in chloride (Cl-), bicarbonate (HCO3-) and mucus transport in rectal biopsies of CF patients - Anion and mucus secretion in CF patients

Status

Recruiting

Phases

Unknown

Study type

Observational

Source

NL-OMON

Registry ID

NL-OMON43631

Enrollment

Registered

2012-04-11

Start date

2014-05-08

Completion date

Unknown

Last updated

2024-08-12

For informational purposes only — not medical advice. Sourced from public registries and may not reflect the latest updates. Terms

Conditions

Cystic Fibrosis Mucoviscidosis

Interventions

None listed

Eligibility

Age

18 Years to 99 Years

Inclusion criteria

Inclusion criteria: For patients: CF (F508del homozygotes) For controls: non-CF, healthy

Exclusion criteria

Exclusion criteria: For patients: non CF For controls: CF or CF carrier

Design outcomes

Primary

Measure	Time frame
HCO3- and mucus transport in native human intestine, and about possible effects of pharmacological correctors on these parameters in CF patients and controls, by measuring: 1. Comparative current measurements in the presence and absence of HCO3- and carbonic anhydrase (Car) inhibitors. 2. pH-stat measurements of total HCO3- secretion to determine wether an electroneutral component contributes tot colonic HCO3- secretion. 3. Measuring HCO3- transport in the presence of bestrophin-2 inhibitors. 4. Cl-, HCO3- and mucustransport in biopsies of CF patients incubated with CFTR correctors. 5. HCO3- transport in distal colon incubated with PPAR-γ agonists or NHE3 inhibitor.	—

Measure

Time frame

HCO3- and mucus transport in native human intestine, and about possible effects of pharmacological correctors on these parameters in CF patients and controls, by measuring: 1. Comparative current measurements in the presence and absence of HCO3- and carbonic anhydrase (Car) inhibitors. 2. pH-stat measurements of total HCO3- secretion to determine wether an electroneutral component contributes tot colonic HCO3- secretion. 3. Measuring HCO3- transport in the presence of bestrophin-2 inhibitors. 4. Cl-, HCO3- and mucustransport in biopsies of CF patients incubated with CFTR correctors. 5. HCO3- transport in distal colon incubated with PPAR-γ agonists or NHE3 inhibitor.

—

Secondary

Measure	Time frame
Confocal microscopy will be applied to determine possible changes in expression and localization of Best2 in the CF biopsies. Mucus stasis will be assessed by Alcian Blue staining of the biopsies [11,14,17], and expression and (re)distribution of transporters (CFTR, Best2) and enzymes (Car4, Car2) will be examined by immunostaining and confocal microscopy [11,14].	—

Countries

Netherlands

Outcome results