hereditary fructose intolerance
Conditions
Interventions
HFI patients receive once daily (in the morning) three tablets of 100 mg
PF-06835919 for 9 days. They will subsequently be gradually exposed to
increasing doses of either oral fructose or glucose (p
Sponsors
Medisch Universitair Ziekenhuis Maastricht
Eligibility
Age
18 Years to 99 Years
Inclusion criteria
Inclusion criteria: • Participants are able to provide signed and dated written informed consent prior to any study specific procedures • Use of effective contraception (only applicable to premenopausal women) • Aged >= 18 years
Exclusion criteria
Exclusion criteria: • Type 2 diabetes • Patients with congestive heart failure and/or severe renal and/or liver insufficiency • Uncontrolled hypertension • Previous enrolment in a clinical study with an investigational product during the last 3 months or as judged by the investigator which would possibly hamper our study results • Use of drugs that inhibit organic anion transporting polypeptide (OATP) transporters (e.g. rifampicin, gemfibrozil, cyclosporine, erythromcyin and clarithromycin)* • Subjects who do not want to be informed about unexpected medical findings * Exclusion criterion for HFI patients only.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| - Intrahepatic lipid content assessed by proton magnetic resonance spectroscopy (at baseline and completion in HFI patients only), - Intestinal fructose tolerance, i.e. onset of abdominal pain and/or nausea after oral fructose challenge (in comparison to oral glucose challenge) - Hepatic fructose tolerance, i.e. serum glucose and phosphate after oral fructose challenge (in comparison to oral fructose challenge in healthy individuals) - Renal fructose tolerance, i.e. urinary glucose, phosphate, pH and amino acids after oral fructose challenge (in comparison to oral fructose challenge in healthy individuals). | — |
Secondary
| Measure | Time frame |
|---|---|
| - | — |
Countries
Netherlands
Outcome results
None listed