NCT04394286

Brief Summary

The purpose of this study is to evaluate the safety and dose escalation of SHP648 an adeno-associated viral vector for gene transfer in hemophilia B participants.

Trial Health

60
Monitor

Trial Health Score

Automated assessment based on enrollment pace, timeline, and geographic reach

Enrollment
2

participants targeted

Target at below P25 for phase_1

Timeline
Completed

Started May 2020

Geographic Reach
2 countries

2 active sites

Status
terminated

Health score is calculated from publicly available data and should be used for screening purposes only.

Trial Relationships

Click on a node to explore related trials.

Study Timeline

Key milestones and dates

Study Start

First participant enrolled

May 13, 2020

Completed
2 days until next milestone

First Submitted

Initial submission to the registry

May 15, 2020

Completed
4 days until next milestone

First Posted

Study publicly available on registry

May 19, 2020

Completed
12 months until next milestone

Primary Completion

Last participant's last visit for primary outcome

May 3, 2021

Completed
Same day until next milestone

Study Completion

Last participant's last visit for all outcomes

May 3, 2021

Completed
1 year until next milestone

Results Posted

Study results publicly available

May 19, 2022

Completed
Last Updated

May 19, 2022

Status Verified

April 1, 2022

Enrollment Period

12 months

First QC Date

May 15, 2020

Results QC Date

April 27, 2022

Last Update Submit

May 17, 2022

Conditions

Outcome Measures

Primary Outcomes (1)

  • Number of Participants With SHP648 Related Serious and Non- Serious Adverse Events (AEs)

    An AE was defined as any untoward medical occurrence in a participant administered a pharmaceutical product and that did not necessarily have a causal relationship with this investigational product (IP) or medicinal product. A Serious adverse event (SAE) was an AE resulting in any of the following outcomes: death; Life-threatening event; required or prolonged inpatient hospitalization; persistent or significant disability/incapacity; congenital anomaly.

    From study start date to Month 12

Secondary Outcomes (8)

  • Plasma Factor IX (FIX) Levels Before and After SHP648 Infusion

    From study start date to Month 12

  • Annualized Bleed Rate (ABR) Before and After SHP648 Infusion

    From study start date to Month 12

  • Number of Participants With Positive Binding Antibody Titers to Adeno-Associated Virus (AAV8)

    From study start date to Month 12

  • Number of Participants With Positive Neutralizing Antibody Titers to AAV8

    From study start date to Month 12

  • Number of Participants With T-cell Response to AAV8

    From study start date to Month 12

  • +3 more secondary outcomes

Study Arms (3)

Cohort 1

EXPERIMENTAL

Cohort 1 participants will receive a single intravenous (IV) infusion of SHP648 on the day of dosing (Day 0).

Genetic: SHP648

Cohort 2

EXPERIMENTAL

Cohort 2 participants will receive a single IV infusion of SHP648 at a 2 to 3-fold escalation of Cohort 1 on the day of dosing (Day 0).

Genetic: SHP648

Cohort 3

EXPERIMENTAL

Cohort 3 participants will receive a single IV infusion of SHP648 at a 2 to 3-fold escalation of Cohort 2 on the day of dosing (Day 0).

Genetic: SHP648

Interventions

SHP648GENETIC

Participants will receive a single IV infusion of SHP648 in Cohort 1, 2, 3 on Day 0.

Also known as: TAK748, AAV8.ss-3xCRM8-TTR-FIX_R338Lopt
Cohort 1Cohort 2Cohort 3

Eligibility Criteria

Age18 Years - 75 Years
Sexmale
Healthy VolunteersNo
Age GroupsAdult (18-64), Older Adult (65+)

You may qualify if:

  • Male, aged 18 to 75 years at the time of screening.
  • Established severe or moderately severe hemophilia B (plasma FIX activity lesser than or equal to \[\<=\] 2 percent (%) measured following greater than or equal to \[\>=\] 5 half-lives of most recent exposure to exogenous FIX) and either \>= 3 hemorrhages per year requiring treatment with exogenous FIX or use of prophylactic therapy.
  • History of greater than (\>) 50 exposure days to exogenously administered FIX concentrates or cryoprecipitates.
  • Sexually active men must agree to use barrier contraception (combination of a condom and spermicide) or limit sexual intercourse to post-menopausal, surgically sterilized, or contraception-practicing partners for a minimum of 6 months after administration of SHP648, or until SHP648 genomes are no longer detected in the semen (whichever is sooner).
  • Signed informed consent.

You may not qualify if:

  • Bleeding disorder(s) other than hemophilia B.
  • Documented laboratory evidence of having developed inhibitors (\>= 0.6 Bethesda Units \[BU\] on any single test) to FIX proteins at any time.
  • Documented prior allergic reaction to any FIX product.
  • Anti-AAV8 neutralizing antibody titer \>= 1:5.
  • Known hypersensitivity to prednisolone or prednisone, or to any of the excipients.
  • Having a disease in which treatment with prednisolone or prednisone is not tolerated (including, but not limited to osteoporosis with vertebral fractures, severe labile hypertension, and brittle diabetes).
  • Evidence of markers of potential underlying risk for autoimmune mediated hepatic disease:
  • Anti-smooth muscle antibody (ASMA) titer \>= 1:40. Values of 1:31 to 1:39 will be flagged as possibly abnormal and the Investigator and Medical Monitor will evaluate the participant for eligibility
  • Elevated anti-liver-kidney microsomal antibody type 1 (LKM1) titers
  • Total Immunoglobulin G (IgG) \> 1.5x upper limit of normal (ULN)
  • Antinuclear antibody (ANA) titer \> 1:320 OR ANA titer \> 1:80 if demonstrated concurrently with alanine aminotransferase (ALT) that is \> ULN
  • Active Hepatitis C: as indicated by detectable hepatitis C virus ribonucleic acid (HCV RNA) by polymerase chain reaction (PCR).
  • Hepatitis B: If surface hepatitis B virus (HBV) antigen is positive.
  • Receiving chronic systemic antiviral and/or interferon therapy within 4 weeks prior to enrollment.
  • Clinically significant infections (e.g., systemic fungal infections) requiring systemic treatment.
  • +25 more criteria

Contact the study team to confirm eligibility.

Sponsors & Collaborators

Study Sites (2)

Hospital Universitario Virgen de la Arrixaca

El Palmar, 30120, Spain

Location

Ege University Medical Faculty

Izmir, 35100, Turkey (Türkiye)

Location

Related Links

MeSH Terms

Conditions

Hemophilia B

Condition Hierarchy (Ancestors)

Blood Coagulation Disorders, InheritedBlood Coagulation DisordersHematologic DiseasesHemic and Lymphatic DiseasesCoagulation Protein DisordersHemorrhagic DisordersGenetic Diseases, InbornCongenital, Hereditary, and Neonatal Diseases and AbnormalitiesGenetic Diseases, X-Linked

Limitations and Caveats

The study was terminated by the Sponsor. Only 2 participants were screened and signed informed consent but none received any treatment during this study. No participants were evaluated, and no data was collected to be reported here, in order to protect and maintain participant's privacy/confidentiality.

Results Point of Contact

Title
Study Director
Organization
Shire

Study Officials

  • Study Director

    Shire

    STUDY DIRECTOR

Publication Agreements

PI is Sponsor Employee
No
Restriction Type
OTHER
Restrictive Agreement
Yes

Study Design

Study Type
interventional
Phase
phase 1
Allocation
NON RANDOMIZED
Masking
NONE
Purpose
TREATMENT
Intervention Model
SEQUENTIAL
Sponsor Type
INDUSTRY
Responsible Party
SPONSOR

Study Record Dates

First Submitted

May 15, 2020

First Posted

May 19, 2020

Study Start

May 13, 2020

Primary Completion

May 3, 2021

Study Completion

May 3, 2021

Last Updated

May 19, 2022

Results First Posted

May 19, 2022

Record last verified: 2022-04

Data Sharing

IPD Sharing
Will not share

De-identified individual participant data from this particular study will not be shared as there is a reasonable likelihood that individual patients could be re-identified (due to the limited number of study participants/study sites)

Locations