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A Phase 2 Study of ELX-02 in Patients With Nephropathic Cystinosis

Phase 2
Terminated
Conditions
Genetic Disease
Cystinosis
Nonsense Mutation
Interventions
Registration Number
NCT04069260
Lead Sponsor
Eloxx Pharmaceuticals, Inc.
Brief Summary

This is a Phase 2 open label study to evaluate the safety, tolerability, PK, and PD of multiple dose levels of SC administered ELX-02 in patients with cystinosis with nonsense mutation in at least one allele.

Six patients will be enrolled in the trial.

The study will comprise of the following periods for each patient:

* A screening period of up to 6 weeks

* A total treatment period of 4 weeks

* A safety follow-up period of 4 weeks after the last treatment

Each patient will receive three escalating doses as follows:

* Treatment period 1: ELX-02 0.5 mg/kg SC daily for 7 days (total dose not to exceed 3.5 mg/kg for this week; the daily dose will be individualized to achieve the target weekly exposure of about 47.5 µg\*h/mL)

* Treatment period 2: ELX-02 1.0 mg/kg SC daily for 7 days (total dose not to exceed 7.0 mg/kg for this week; the daily dose will be individualized to achieve the target weekly exposure of about 95 µg\*h/mL)

* Treatment period 3: ELX-02 2.0 mg/kg SC daily for 14 days (total dose not to exceed 14 mg/kg for these two weeks; the daily dose will be individualized to achieve the target weekly exposure of about 190 µg\*h/mL)

Detailed Description

Not available

Recruitment & Eligibility

Status
TERMINATED
Sex
All
Target Recruitment
3
Inclusion Criteria

Not provided

Exclusion Criteria

Not provided

Study & Design

Study Type
INTERVENTIONAL
Study Design
SINGLE_GROUP
Arm && Interventions
GroupInterventionDescription
ELX-02ELX-02Eukaryotic ribosomal selective glycoside (ERSG)
Primary Outcome Measures
NameTimeMethod
Maximum observed plasma concentration (Cmax)Day 1 of treatment periods 1, 2, and 3

Full PK profile 12 blood samples over 24 hours

AEs associated with different dose levels of ELX-02From the time of first dosing through the follow-up visit, an average of approximately 10 weeks
Observed plasma concentration at 1 hour post dose (C1h)Days 1, 2, 5, and 7 of treatment periods 1-2; Days 1, 2, 5, 7, 10, and 14 of treatment period 3

Sparse sampling, blood sampling only, pre-dose and 1 hour post dose

Amount of ELX-02 excreted in urine from 0 to 24 hours (Ae24h)Day 1 of treatment periods 1, 2, and 3

6 urine collections over 24 hours

Renal clearance on Day 1 (Ae24h/plasma AUC0-24h)Day 1 of treatment periods 1, 2, and 3

6 urine collections over 24 hours

Area under the plasma concentration curve from time zero to 24 hours (AUC0-24h)Day 1 of treatment periods 1, 2, and 3

Full PK profile 12 blood samples over 24 hours

Secondary Outcome Measures
NameTimeMethod
Changes from baseline in WBC cystine levelsScreening; Days 1, 2, 5, and 7 of treatment periods 1-2; Days 1, 2, 5, 7, 10, and 14 of treatment period 3, and 4-week safety follow-up

Trial Locations

Locations (1)

McGill University Health Center

🇨🇦

Montréal, Quebec, Canada

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