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An open-label phase 3 study of lucerastat in Japanese subjects with Fabry disease

Phase 3
Completed
Conditions
Fabry disease
Registration Number
JPRN-jRCT2080224659
Lead Sponsor
Idorsia Pharmaceuticals Japan Ltd
Brief Summary

Not available

Detailed Description

Not available

Recruitment & Eligibility

Status
completed
Sex
All
Target Recruitment
20
Inclusion Criteria

(1)Patients who have given consent to participate in this study.
(2)Male and female Japanese patients of at least 16 years of age
(3)Patients who have received a final diagnosis of Fabry disease confirmed by a genetic test (patients found to have at least 1 genetic abnormality in the GLA gene coding alpha-galactosidase A [alpha-GalA])
(4)Patients with history of treatment for Fabry disease that meets the following:
a)Lucerastat monotherapy group (One of the following conditions has to apply)
-Patient has not received ERT for at least 6 months at the time of the consent
-Patients receiving treatment with ERT at the time of consent who wishes to be treated with the study drug and agrees to discontinue the current treatment with existing drugs before the start of study drug administration
b)ERT-lucerastat combination therapy group
-Patients treated with ERT for at least 12 months at the time of consent
(5)In case of women who have a potential of childbearing, meeting all the following conditions:
-Patients with negative results from serum pregnancy test during the screening period (V1).
-Patients who consent to undertake monthly urine pregnancy tests during the period up to EOS.
-Patients who consent to comply with a method of contraception scheme in the protocol during the period up to EOS.
(6)Male patients with reproductive potential (in the opinion of the principal investigator or subinvestigator) meeting the two following conditions:
-Patients who agree to using a condom during the period up to EOS (84 days (+7 days) after EOT).
-Patients who agree to refrain from attempting to father a child during the period up to EOS (84 days (+7 days) after EOT)
(7)Patients who agree to record patient diary during the period from informed consent until EOT.

Exclusion Criteria

(1)Patients who become pregnant, plan to become pregnant or breastfeed, during the period up to EOS.
(2)Patients with severe renal impairment with an estimated glomerular filtration rate (eGFR) < 30 mL/min/1.73 m2 calculated based on the laboratory test results performed during the screening period.
(3)Patients receiving renal dialysis
(4)Patients with a transplanted kidney or other organ
(5)Patients with a history of transient ischemic attack, stroke, unstable angina pectoris or myocardial infarction within 6 months before the consent.
(6)Patients with unstable heart disease as judged by the principal investigator or subinvestigator (e.g., uncontrolled arrhythmia or NYHA class III or IV heart failure).
(7)Patients with a risk of worsening of clinical symptoms due to an organ lesion during the study period in the opinion of the principal investigator or subinvestigator
(8)Patients with the following causal factors or disease causing difficulty complying with the study drug regimen, or difficulty conducting the study or interpreting results
(a)Patients with disease conditions hindering evaluation of neuropathic pain (for example: diabetic neuropathy, peripheral neuropathy due to chemotherapy or radiotherapy, chronic inflammatory demyelinating neuropathy, etc.)
(b)Patients with other diseases hindering the evaluation of gastrointestinal symptoms due to Fabry disease
(c)Patients with uncontrollable diabetes mellitus (for example: patients with HbA1c > 8.0% measured during the screening period)
(d)patients with obvious nervous disease
(e)Patients with obvious psychiatric disease (including suicidal ideation during the screening period or suicide attempt/suicidal behavior within 6 months before consent is obtained in the opinion of the principal investigator or subinvestigator)
(f)Patients with a history of drug dependence (including opioids) or alcohol dependence
(g)Patients with difficulty recording patient diaries every day
(9)Patients with a fatal disease with life expectancy < 18 months
(10)Patients with hypersensitivity to the study drug or any drugs in the same class of imino sugars (for example: miglitol, miglustat or migalastat) or their excipients
(11)Patients who have started any of the following drugs, or had the dosage of any of the following drugs changed, within 4 weeks before consent was obtained
(a)Angiotensin converting enzyme (ACE) inhibitors and/or angiotensin receptor blockers (ARBs)
(b)adjuvant analgesics (Antiepileptics, TCA and SNRI/SSRI) and/or other antidepressants)
(12)Patients currently receiving other study drugs, or who have received other study drug within 3 months before consent was obtained (for example, lucerastat, ibiglustat, venglustat)
(13)Patients who have received treatment with glucosylceramide synthase (GCS) inhibitors (e.g., miglustat, eliglustat) or an alpha-GalA chaperone (migalastat) within the last 6 months before the consent.
(14)Other patients who are considered ineligible for the study subject in the opinion of the principal investigator or subinvestigator (expected difficulty of study visit or drug compliance etc.)

Study & Design

Study Type
Interventional
Study Design
Not specified
Primary Outcome Measures
NameTimeMethod
efficacy<br>Change in plasma Gb3 from baseline to end of treatment
Secondary Outcome Measures
NameTimeMethod
safety<br>pharmacokinetics<br>Safety: Treatment emergent adverse event and serious adverse event<br>Pharmacokinetics: blood drug concentration
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