Ketohexokinase Inhibition in Hereditary Fructose Intolerance

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; a pregnancy test will be performed in these women at baseline) * Aged ≥ 18 years Exclusion Criteria: * Diabetes mellitus * Pregnancy * 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 B1 (OATPB1) transporters (e.g. rifampicin, gemfibrozil, ciclosporine, erythromcyin and clarithromycin)\* * Treatment with irinotecan\* Any contra-indications for MRI scanning\* * Subjects who do not want to be informed about unexpected medical findings * Exclusion criterion for HFI patients only.