Application of zileuton (Zyflo CR) in patients with Sjögren Larsson Syndrome - Zileuton in Sjögren Larsson Syndrome

Status

Active, not recruiting

Phases

Phase 3

Study type

Interventional

Source

EU CTR

Registry ID

EUCTR2009-015895-87-NL

Enrollment

Registered

2009-10-01

Start date

2009-12-23

Completion date

Unknown

Last updated

2022-05-30

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

Conditions

Sjögren Larsson syndrome (SLS) is an autosomal recessive inherited neurometabolic disorder which is characterized by a clinical triad of congenital ichthyosis, spastic di- or tetraplegia and mental retardation. A disturbance of lipid metabolism due to deficiency of the microsomal fatty aldehyde dehydrogenase (FALDH) underlies SLS.

Interventions

Trade Name: Zyflo CR Product Name: zileuton Pharmaceutical Form: Tablet INN or Proposed INN: ZILEUTON CAS Number: 111406872 Other descriptive name: lipoxygenase inhibitor Concentration unit: mg milli

Eligibility

Sex/Gender

All

Inclusion criteria

Inclusion criteria: Biochemical and genetically proven Sjögren Larsson patients. Age > 6 years. Parental informed consent. Are the trial subjects under 18? yes Number of subjects for this age range: F.1.2 Adults (18-64 years) yes F.1.2.1 Number of subjects for this age range F.1.3 Elderly (>=65 years) no F.1.3.1 Number of subjects for this age range

Exclusion criteria

Exclusion criteria: Renal and/or hepatic failure Use of alcohol. Smoking. Use of theophylline, warfarin and/or propranolol during the study period. Use of zileuton less than 6 weeks prior to start of the study. Patients not willing to maintain their dermatological treatment stable during the study period. Pregnancy and/or lactation.

Design outcomes

Primary

Measure	Time frame
Main Objective: To investigate the effect of Zileuton on the clinical parameters pruritus, ichthyosis as well as on the urinary concentrations of LTB4 and its metabolite omega-OH-LTB4.;Secondary Objective: Secondary, we study pharmacokinetic and pharmacodynamic properties of zileuton in humans as well as the dosage-effect relationship.;Primary end point(s): Clinical endpoints: Decrease in severity of pruritus and excoriations as well as improvement of ichthyosis measured by visual analogue scores (VAS), “Willemsen score” (both filled out by parents/caregivers) as well as the clinical Sjögren Larsson Area Severity Index (SLASI). Decrease in the amount of topical emollients that is needed by the patients. Biochemical endpoints: Significant decrease in urinary LTB4 and 20-OH-LTB4 metabolite concentrations of SLS patients during treatment with zileuton.	—

Measure

Time frame

Main Objective: To investigate the effect of Zileuton on the clinical parameters pruritus, ichthyosis as well as on the urinary concentrations of LTB4 and its metabolite omega-OH-LTB4.;Secondary Objective: Secondary, we study pharmacokinetic and pharmacodynamic properties of zileuton in humans as well as the dosage-effect relationship.;Primary end point(s): Clinical endpoints: Decrease in severity of pruritus and excoriations as well as improvement of ichthyosis measured by visual analogue scores (VAS), “Willemsen score” (both filled out by parents/caregivers) as well as the clinical Sjögren Larsson Area Severity Index (SLASI). Decrease in the amount of topical emollients that is needed by the patients. Biochemical endpoints: Significant decrease in urinary LTB4 and 20-OH-LTB4 metabolite concentrations of SLS patients during treatment with zileuton.

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Countries

Netherlands

Outcome results

None listed

Source: EU CTR (via WHO ICTRP) · Data processed: Feb 4, 2026