Phase I/II Clinical Study to Evaluate the Safety, Tolerability and Efficacy of LY-M003 Injection in Adult Patients With Wilson's Disease
A Multicenter, Open, Single-arm, Single-dose, Phase I/II Study Evaluating the Safety, Tolerability, and Efficacy of LY-M003 Injection in Adult Patients With Wilson's Disease
Studienübersicht
Status
Status
Bedingungen
Bedingungen
Intervention / Behandlung
Intervention / Behandlung
Detaillierte Beschreibung
This is a multicenter, open-label, single-arm, single-dose Phase I/II clinical study. It aims to evaluate the safety, tolerability, efficacy, immunogenicity, pharmacodynamic (PD) and pharmacokinetic (PK) profiles of LY-M003 Injection in patients with Wilson's Disease (WD). The Phase I/II study consists of a main study phase and a long-term follow-up phase. The main study phase includes an 8-week screening period and a 52-week follow-up period after infusion. The long-term follow-up phase starts at Week 53 and lasts until 5 years post-infusion.
The study pre-specified two dose cohorts, consisting of one main dose cohort and one de-escalation dose cohort: Dose Cohort 1 (4.0 × 10¹³ vg/kg) and the de-escalation dose cohort (2.0 × 10¹³ vg/kg). Three subjects will be enrolled in each dose cohort.Dose Cohort 1 serves as the starting dose. After the first subject receives the study drug, a minimum 28-day DLT observation period must be completed to confirm safety before enrolling subsequent subjects.All three subjects in Dose Cohort 1 have completed the 28-day (Day 28) DLT observation period following LY-M003 infusion. The Safety Review Committee (SRC) will make a comprehensive assessment to determine whether to proceed to the dose expansion phase at the dose level of 4.0 × 10¹³ vg/kg.
Studientyp
Studientyp
Einschreibung (Geschätzt)
Einschreibung
Phase
Phase
- Phase 2
- Phase 1
Kontakte und Standorte
Studienkontakt
Studienkontakt
- Name: Rihong He
- Telefonnummer: +8619121572057
- E-Mail: rihong.he@lingyimed.com
Studienorte
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Zhejiang
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Hangzhou, Zhejiang, China, 310003
- The First Affiliated Hospital, Zhejiang University School of Medicine
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Hauptermittler:
- Chaohui Yu, PhD
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Kontakt:
- Chaohui YU, PhD
- Telefonnummer: +86 13957161659
- E-Mail: ych623@sina.com
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Teilnahmekriterien
Zulassungskriterien
Zulassungskriterien
Studienberechtigtes Alter
- Erwachsene
Akzeptiert gesunde Freiwillige
Beschreibung
Inclusion Criteria:
- The subject fully comprehends the purpose, design, methods and possible adverse events of the study, agrees to participate voluntarily and signs the informed consent form (ICF).
- Patients with confirmed diagnosis of Wilson's disease (WD).
- Subjects with Wilson's disease (WD) confirmed by laboratory testing to have biallelic ATP7B gene mutation or deletion.
- The subjects are treated patients with Wilson's disease (WD) who have received standard therapy (e.g., D-penicillamine or zinc acetate) continuously for at least 6 months prior to screening.
- Subjects have maintained a low-copper diet for at least 6 consecutive months prior to screening and will continue this dietary restriction throughout the study.
- Subjects must agree to refrain from donating blood, organs, tissues or cells at any time after treatment.
- Female subjects of childbearing potential (WOCBP) must have a negative pregnancy test.
- Subjects and their partners must have no plans for pregnancy from screening through 6 months after study completion, and will voluntarily use effective contraception (e.g., abstinence, condoms). Subjects shall not plan to donate sperm or ova.
Exclusion Criteria:
- AAV8 neutralizing antibody titer > 1:10 .
- History of active gastrointestinal bleeding within the past 3 months.
- Decompensated liver cirrhosis or advanced liver disease presenting with portal hypertension, ascites, splenomegaly, esophageal varices, hepatic encephalopathy, etc.
- Subjects with other concomitant liver diseases as judged by the investigator, including autoimmune hepatitis, alcoholic liver disease, primary biliary cholangitis, primary sclerosing cholangitis, and/or drug- or toxin-induced liver disease.
- Subjects with severe hypersplenism complicated and requiring splenectomy as assessed by the investigator.
- Model for End-Stage Liver Disease (MELD) score > 13.
- Other disorders of copper metabolism, such as chronic cholestatic liver diseases, disorders of glycosylation, copper metabolism disorders, etc.
- A history of non-compliance with copper chelators or zinc agents as assessed by the investigator within 6 months prior to screening.
- Previously treated WD subjects with ALT and/or AST levels more than 5 times the upper limit of normal (ULN).
- Subjects with severe neurological deficits or impairments that, in the investigator's judgment, compromise their safety and/or ability to participate in the study.
- Hemoglobin < 90g/L.
- Subjects with positive hepatitis B surface antigen (HBsAg), positive hepatitis C virus (HCV) antibody, positive human immunodeficiency virus (HIV) antibody or positive treponema pallidum antibody.
- Subjects with end-stage renal disease on dialysis (Chronic Kidney Disease Stage 3 and above), or creatinine clearance < 60 mL/min.
- Severe hyperlipidemia (triglycerides >1000 mg/dL);
- Subjects who have received or plan to undergo bone marrow transplantation, hematopoietic stem cell transplantation and/or major organ transplantation, including but not limited to liver transplantation and renal transplantation.
- Subjects with clinically diagnosed severe cardiovascular diseases or those deemed by the investigator to have such conditions (e.g., New York Heart Association [NYHA] heart failure classification ≥ Class 3).
- Subjects with uncontrolled concomitant diseases or infectious diseases as assessed by the investigator.
- Subjects who are allergic to any ingredient of LY-M003 Injection.
- Prior receipt of any type of gene therapy or cell therapy.
- Use of systemic immunosuppressants or steroids within 3 months prior to administration (except for prophylactic immunosuppressive therapy specified in the protocol).
- History of cancer within 5 years prior to screening, excluding completely resected non-melanoma skin cancer, non-metastatic prostate cancer and fully cured ductal carcinoma in situ.
- Received live attenuated vaccines within 4 months prior to screening, or planned to receive such vaccines during the clinical trial.
- Received treatment or intervention with other investigational drugs or study devices within 28 days or 5 half-lives (for drugs only) prior to screening, whichever is longer.
- Pregnant women (or women planning pregnancy) or breastfeeding women.
- Other conditions that, in the investigator's opinion, render the subject ineligible for study participation.
Studienplan
Wie ist die Studie aufgebaut?
Designdetails
- Hauptzweck: Behandlung
- Zuteilung: Nicht randomisiert
- Interventionsmodell: Sequenzielle Zuweisung
- Maskierung: Keine (Offenes Etikett)
Anzahl der Arme
Waffen und Interventionen
Teilnehmergruppe / ArmTeilnehmergruppe / Arm |
Intervention / BehandlungIntervention / Behandlung |
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Experimental: Phase 1: LY-M003 Dose group 1
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Phase 1: Participants receive a single, peripheral intravenous (IV) infusion of LY-M003 at dose group 1.The dose for Dose Group 1 is 4.0 × 10¹³ vg/kg.All participants are administered the drug once via intravenous injection.
Phase 1: Participants receive a single, peripheral intravenous (IV) infusion of LY-M003 at de-escalation dose.The dose for de-escalation dose is 2.0 × 10¹³ vg/kg.All participants are administered the drug once via intravenous injection.
Phase 2: Participants receive a single, peripheral intravenous (IV) infusion of LY-M003 at dose group 1.The dose for dose group 1 is 4.0 × 10¹³ vg/kg.All participants are administered the drug once via intravenous injection.
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Experimental: Phase 1: LY-M003 de-escalation dose
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Phase 1: Participants receive a single, peripheral intravenous (IV) infusion of LY-M003 at dose group 1.The dose for Dose Group 1 is 4.0 × 10¹³ vg/kg.All participants are administered the drug once via intravenous injection.
Phase 1: Participants receive a single, peripheral intravenous (IV) infusion of LY-M003 at de-escalation dose.The dose for de-escalation dose is 2.0 × 10¹³ vg/kg.All participants are administered the drug once via intravenous injection.
Phase 2: Participants receive a single, peripheral intravenous (IV) infusion of LY-M003 at dose group 1.The dose for dose group 1 is 4.0 × 10¹³ vg/kg.All participants are administered the drug once via intravenous injection.
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Experimental: Phase 2: LY-M003 Dose group 1
|
Phase 1: Participants receive a single, peripheral intravenous (IV) infusion of LY-M003 at dose group 1.The dose for Dose Group 1 is 4.0 × 10¹³ vg/kg.All participants are administered the drug once via intravenous injection.
Phase 1: Participants receive a single, peripheral intravenous (IV) infusion of LY-M003 at de-escalation dose.The dose for de-escalation dose is 2.0 × 10¹³ vg/kg.All participants are administered the drug once via intravenous injection.
Phase 2: Participants receive a single, peripheral intravenous (IV) infusion of LY-M003 at dose group 1.The dose for dose group 1 is 4.0 × 10¹³ vg/kg.All participants are administered the drug once via intravenous injection.
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Was misst die Studie?
Primäre Ergebnismessungen
Primäre Ergebnismessungen
Ergebnis Maßnahme |
Maßnahmenbeschreibung |
Zeitfenster |
|---|---|---|
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Phase I:The incidence of dose-limiting toxicity (DLT) events adjudicated by the Safety Review Committee (SRC) within at least 28 days after LY-M003 infusion.
Zeitfenster: Within 28 days after infusion of LY-M003 Injection
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The incidence of DLT will be assessed using CTCAE 6.0.
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Within 28 days after infusion of LY-M003 Injection
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The incidence of treatment-emergent adverse events (AEs), adverse events of special interest (AESIs) and serious adverse events (SAEs) related to LY-M003 within 52 weeks after infusion.
Zeitfenster: 5 years
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Assessment will be conducted via 12-lead ECG, laboratory tests(blood routine, blood biochemistry, coagulation function, stool routine, urine routine), vital signs (blood pressure, pulse, respiratory rate, body temperature) and physical examination.
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5 years
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Sekundäre Ergebnismessungen
Sekundäre Ergebnismessungen
Ergebnis Maßnahme |
Maßnahmenbeschreibung |
Zeitfenster |
|---|---|---|
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Percentage reduction in the dosage of standard of care (SoC) medications within 52 weeks after administration.
Zeitfenster: Within 52 weeks after administration
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Assessment will be based on the percentage of participants who underwent standard dose reduction after infusion.
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Within 52 weeks after administration
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Number and proportion of subjects who discontinued standard of care (SoC) medications within 52 weeks after administration.
Zeitfenster: Within 52 weeks after administration
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Assessment will be based on the number and percentage of participants who discontinued standard treatment medications.
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Within 52 weeks after administration
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Duration (in treatment weeks) of consecutive discontinuation of SoC medications among the above subjects.
Zeitfenster: Within 52 weeks after administration
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Assessment will be based on the number of consecutive cycles during which participants discontinued standard medications.
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Within 52 weeks after administration
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Change in serum non-caeruloplasmin-bound copper (NCC) from baseline within 52 weeks after administration
Zeitfenster: Within 52 weeks after administration
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Assessment will be based on changes in serum non-ceruloplasmin-bound copper from baseline.
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Within 52 weeks after administration
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Change in serum ceruloplasmin level from baseline within 52 weeks after administration
Zeitfenster: Within 52 weeks after administration
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Assessment will be based on changes in serum ceruloplasmin level from baseline.
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Within 52 weeks after administration
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Change in serum ceruloplasmin activity level from baseline within 52 weeks after administration
Zeitfenster: Within 52 weeks after administration
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Assessment will be based on changes in erum ceruloplasmin activity level from baseline.
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Within 52 weeks after administration
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Change in 24-hour urinary copper content from baseline at each follow-up time point within 52 weeks after administration
Zeitfenster: Within 52 weeks after administration
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Assessment will be based on changes in 24-hour urinary copper content from baseline.
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Within 52 weeks after administration
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Change in serum total copper from baseline within 52 weeks after administration
Zeitfenster: Within 52 weeks after administration
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Assessment will be based on changes in serum total copper from baseline
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Within 52 weeks after administration
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Change from baseline in total score of the neurological subscale of the Unified Wilson's Disease Rating Scale (UWDRS) within 52 weeks after administration
Zeitfenster: Within 52 weeks after administration
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Assess the change from baseline in the total score of Part I of the Unified Wilson's Disease Rating Scale (UWDRS).
Part I of the UWDRS is the neurological subscale, with a total score ranging from 0 to 208.
Higher scores indicate more severe disease and poorer functional status.
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Within 52 weeks after administration
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Change from baseline in hepatic subscale score of the Unified Wilson's Disease Rating Scale (UWDRS) within 52 weeks after administration
Zeitfenster: Within 52 weeks after administration
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Assess the change from baseline in the total score of Part II of the Unified Wilson's Disease Rating Scale (UWDRS).
Part II is the hepatic subscale, with a total score ranging from 0 to 36.
Higher scores indicate more severe disease and poorer functional status.
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Within 52 weeks after administration
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Change from baseline in psychiatric subscale score of the Unified Wilson's Disease Rating Scale (UWDRS) within 52 weeks after administration
Zeitfenster: Within 52 weeks after administration
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Assess the change from baseline in the total score of Part III of the Unified Wilson's Disease Rating Scale (UWDRS).
Part III is the psychiatric subscale, with a total score ranging from 0 to 76. Higher scores indicate more severe disease and poorer functional status.
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Within 52 weeks after administration
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Change from baseline in individual item/subscale scores (speech, handwriting, standing and gait) of the Unified Wilson's Disease Rating Scale (UWDRS) within 52 weeks after administration
Zeitfenster: Within 52 weeks after administration
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Assessment will be based on changes from baseline in individual item/subscale scores (speech, handwriting, standing and gait) of the Unified Wilson's Disease Rating Scale (UWDRS).The total score for speech, writing, standing and gait each ranges from 0 to 4. Higher scores indicate more severe conditions.
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Within 52 weeks after administration
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Change in ALT and AST from baseline within 52 weeks after administration
Zeitfenster: Within 52 weeks after administration
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Assessment will be based on changes in ALT and AST from baseline
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Within 52 weeks after administration
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Change in liver stiffness from baseline within 52 weeks after administration
Zeitfenster: Within 52 weeks after administration
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Assessment will be based on changes in liver stiffness from baseline.Liver stiffness is measured by liver and gallbladder ultrasonography combined with ultrasound elastography, with the unit of kPa.
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Within 52 weeks after administration
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Change in Kayser-Fleischer (K-F) rings from baseline within 52 weeks after administration
Zeitfenster: within 52 weeks after administration
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Assessment will be based on changes in Kayser-Fleischer (K-F) rings from baseline
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within 52 weeks after administration
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Long-term follow-up study: Percentage reduction in standard of care (SoC) medication dosage after 52 weeks of administration
Zeitfenster: Weeks 53 to 5 years after LY-M003 infusion
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Assessment will be based on the percentage of participants who underwent standard dose reduction after infusion
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Weeks 53 to 5 years after LY-M003 infusion
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Long-term follow-up study: Number and proportion of subjects who discontinued standard of care (SoC) medications after 52 weeks of treatment
Zeitfenster: Weeks 53 to 5 years after LY-M003 infusion
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Assessment will be based on the number and percentage of participants who discontinued standard treatment medications.
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Weeks 53 to 5 years after LY-M003 infusion
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Long-term follow-up study: Number of consecutive treatment weeks during which the above-mentioned subjects remained off SoC medications
Zeitfenster: Weeks 53 to 5 years after LY-M003 infusion
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Assessment will be based on the number of consecutive cycles during which participants discontinued standard medications.
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Weeks 53 to 5 years after LY-M003 infusion
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Long-term follow-up study: Changes in serum ceruloplasmin activity levels from baseline after 52 weeks of treatment
Zeitfenster: Weeks 53 to 5 years after LY-M003 infusion
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Assessment will be based on changes in erum ceruloplasmin activity level from baseline.
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Weeks 53 to 5 years after LY-M003 infusion
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Long-term follow-up study: Changes in serum ceruloplasmin concentration levels from baseline following 52 weeks of treatment
Zeitfenster: Weeks 53 to 5 years after LY-M003 infusion
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Assessment will be based on changes in serum ceruloplasmin level from baseline.
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Weeks 53 to 5 years after LY-M003 infusion
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Long-term follow-up study: Changes in non-ceruloplasmin bound copper (NCC) in serum from baseline after 52 weeks of treatment
Zeitfenster: Weeks 53 to 5 years after LY-M003 infusion
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Assessment will be based on changes in serum non-ceruloplasmin-bound copper from baseline
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Weeks 53 to 5 years after LY-M003 infusion
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Long-term follow-up study: Changes in total serum copper from baseline after 52 weeks of treatment
Zeitfenster: Weeks 53 to 5 years after LY-M003 infusion
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Assessment will be based on changes in serum total copper from baseline
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Weeks 53 to 5 years after LY-M003 infusion
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Long-term follow-up study: Changes of 24-hour urinary copper levels from baseline at all follow-up visits after 52 weeks of treatment
Zeitfenster: Weeks 53 to 5 years after LY-M003 infusion
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Assessment will be based on changes in 24-hour urinary copper content from baseline.
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Weeks 53 to 5 years after LY-M003 infusion
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Long-term follow-up study: Changes in quantitative neurological function scores of the Unified Wilson's Disease Rating Scale (UWDRS) from baseline after 52 weeks of treatment
Zeitfenster: Weeks 53 to 5 years after LY-M003 infusion
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Assess the change from baseline in the total score of Part I of the Unified Wilson's Disease Rating Scale (UWDRS).
Part I of the UWDRS is the neurological subscale, with a total score ranging from 0 to 208.
Higher scores indicate more severe disease and poorer functional status.
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Weeks 53 to 5 years after LY-M003 infusion
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Long-term follow-up study: Changes in hepatic subscale quantitative scores of the UWDRS from baseline after 52 weeks of treatment
Zeitfenster: Weeks 53 to 5 years after LY-M003 infusion
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Assess the change from baseline in the total score of Part II of the Unified Wilson's Disease Rating Scale (UWDRS).
Part II is the hepatic subscale, with a total score ranging from 0 to 36.
Higher scores indicate more severe disease and poorer functional status.
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Weeks 53 to 5 years after LY-M003 infusion
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Long-term follow-up study: Changes in psychiatric symptom subscale quantitative scores of the UWDRS from baseline after 52 weeks of treatment
Zeitfenster: Weeks 53 to 5 years after LY-M003 infusion
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Assess the change from baseline in the total score of Part III of the Unified Wilson's Disease Rating Scale (UWDRS).
Part III is the psychiatric subscale, with a total score ranging from 0 to 76. Higher scores indicate more severe disease and poorer functional status.
|
Weeks 53 to 5 years after LY-M003 infusion
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Long-term follow-up study: Changes from baseline in individual item and subscale scores (Speech, Handwriting, Standing and Gait) of the quantitative UWDRS after 52 weeks of treatment
Zeitfenster: Weeks 53 to 5 years after LY-M003 infusion
|
Assessment will be based on changes from baseline in individual item/subscale scores (speech, handwriting, standing and gait) of the Unified Wilson's Disease Rating Scale (UWDRS).The total score for speech, writing, standing and gait each ranges from 0 to 4. Higher scores indicate more severe conditions.
|
Weeks 53 to 5 years after LY-M003 infusion
|
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Long-term follow-up study: Changes in ALT and AST levels from baseline after 52 weeks of treatment
Zeitfenster: Weeks 53 to 5 years after LY-M003 infusion
|
Assessment will be based on changes in ALT and AST from baseline
|
Weeks 53 to 5 years after LY-M003 infusion
|
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Long-term follow-up study: Changes in liver stiffness measurements from baseline after 52 weeks of treatment
Zeitfenster: Weeks 53 to 5 years after LY-M003 infusion
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Assessment will be based on changes in liver stiffness from baseline.Liver stiffness is measured by liver and gallbladder ultrasonography combined with ultrasound elastography, with the unit of kPa.
|
Weeks 53 to 5 years after LY-M003 infusion
|
|
Long-term follow-up study: Changes in Kayser-Fleischer (K-F) ring grading from baseline after 52 weeks of treatment
Zeitfenster: Weeks 53 to 5 years after LY-M003 infusion
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Assessment will be based on changes in Kayser-Fleischer (K-F) rings from baseline
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Weeks 53 to 5 years after LY-M003 infusion
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Mitarbeiter und Ermittler
Sponsor
Sponsor
Ermittler
Ermittler
- Hauptermittler: Chaohui Yu, PhD, Zhejiang University
Studienaufzeichnungsdaten
Haupttermine studieren
Studienbeginn (Geschätzt)
Studienbeginn
Primärer Abschluss (Geschätzt)
Primärer Abschluss
Studienabschluss (Geschätzt)
Studienabschluss
Studienanmeldedaten
Zuerst eingereicht
Zuerst eingereicht
Zuerst eingereicht, das die QC-Kriterien erfüllt hat
Zuerst eingereicht, das die QC-Kriterien erfüllt hat
Zuerst gepostet (Tatsächlich)
Zuerst gepostet
Studienaufzeichnungsaktualisierungen
Letztes Update gepostet (Tatsächlich)
Letztes Update gepostet
Letztes eingereichtes Update, das die QC-Kriterien erfüllt
Letztes eingereichtes Update, das die QC-Kriterien erfüllt
Zuletzt verifiziert
Zuletzt verifiziert
Mehr Informationen
Begriffe im Zusammenhang mit dieser Studie
Schlüsselwörter
Zusätzliche relevante MeSH-Bedingungen
- Erkrankungen des Gehirns
- Erkrankungen des zentralen Nervensystems
- Erkrankungen des Nervensystems
- Stoffwechsel, angeborene Fehler
- Genetische Krankheiten, angeboren
- Stoffwechselerkrankungen
- Erkrankungen des Verdauungssystems
- Neurodegenerative Krankheiten
- Leberkrankheiten
- Bewegungsstörungen
- Heredodegenerative Erkrankungen, Nervensystem
- Erkrankungen der Basalganglien
- Gehirnerkrankungen, Stoffwechselerkrankungen, angeboren
- Gehirnerkrankungen, Stoffwechsel
- Metallstoffwechsel, angeborene Fehler
- Angeborene, erbliche und neonatale Krankheiten und Anomalien
- Ernährungs- und Stoffwechselerkrankungen
- Hepatolentikuläre Degeneration
Andere Studien-ID-Nummern
Andere Studien-ID-Nummern
- LY-M003-WD-002
Plan für individuelle Teilnehmerdaten (IPD)
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