Lonafarnib for Patients With Hutchinson-Gilford Progeria Syndrome or Progeroid Laminopathy
Primary Purpose
Progeria, HGPS
Status
Approved for marketing
Phase
Locations
Study Type
Expanded Access
Intervention
Lonafarnib
Sponsored by
About this trial
This is an expanded access trial for Progeria
Eligibility Criteria
Inclusion Criteria:
- Clinical diagnosis of HGPS or progeroid laminopathy by qualified medical doctor (based on common phenotype as described in Gordon 2015 and Merideth 2008). Confirmation with genetic testing is preferred but not required.
- Adequate hepatic function as defined by SGPT (ALT) and SGOT (AST) ≤ 5 times upper limit of normal range for age
Exclusion Criteria:
- Taking medications or foods that are known to be moderate or strong inducers or inhibitors of CYP3A4 or sensitive CYP3A substrates; or if a patient is taking one of these drugs and cannot safely discontinue or take an alternative drug, the dose of the inhibitor/inducer must be adjusted per the treating physician
- Taking digoxin, a P-gp substrate with a narrow therapeutic window.
- Severe renal impairment (GFR < 30 mL/min/1.73m2).
- Uncontrolled infection.
- Presence of any active clinically relevant medical condition that in the opinion of the treating physician would preclude patient from safely participating in the program.
- Pregnant or breast-feeding or plan to become pregnant while on therapy.
Sites / Locations
Outcomes
Primary Outcome Measures
Secondary Outcome Measures
Full Information
NCT ID
NCT03895528
First Posted
March 27, 2019
Last Updated
April 13, 2021
Sponsor
Eiger BioPharmaceuticals
1. Study Identification
Unique Protocol Identification Number
NCT03895528
Brief Title
Lonafarnib for Patients With Hutchinson-Gilford Progeria Syndrome or Progeroid Laminopathy
Official Title
A Treatment IND (Investigational New Drug) Protocol for EAP (Expanded Access Program) for the Use of Lonafarnib in Patients With Hutchinson-Gilford Progeria Syndrome (HGPS) or Progeroid Laminopathy
Study Type
Expanded Access
2. Study Status
Record Verification Date
April 2021
Overall Recruitment Status
Approved for marketing
Study Start Date
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Primary Completion Date
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Study Completion Date
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3. Sponsor/Collaborators
Responsible Party, by Official Title
Sponsor
Name of the Sponsor
Eiger BioPharmaceuticals
4. Oversight
5. Study Description
Brief Summary
This treatment IND protocol will allow patients with HGPS and progeroid laminopathies access to lonafarnib, the only compound shown to have an effect on the HGPS disease process resulting in improved outcomes (Gordon et al, 2018). There are no approved treatments for HGPS and progeroid laminopathies.
6. Conditions and Keywords
Primary Disease or Condition Being Studied in the Trial, or the Focus of the Study
Progeria, HGPS
7. Study Design
8. Arms, Groups, and Interventions
Intervention Type
Drug
Intervention Name(s)
Lonafarnib
Other Intervention Name(s)
FTI
Intervention Description
Farnesyl transferase inhibitor
10. Eligibility
Sex
All
Minimum Age & Unit of Time
12 Months
Eligibility Criteria
Inclusion Criteria:
Clinical diagnosis of HGPS or progeroid laminopathy by qualified medical doctor (based on common phenotype as described in Gordon 2015 and Merideth 2008). Confirmation with genetic testing is preferred but not required.
Adequate hepatic function as defined by SGPT (ALT) and SGOT (AST) ≤ 5 times upper limit of normal range for age
Exclusion Criteria:
Taking medications or foods that are known to be moderate or strong inducers or inhibitors of CYP3A4 or sensitive CYP3A substrates; or if a patient is taking one of these drugs and cannot safely discontinue or take an alternative drug, the dose of the inhibitor/inducer must be adjusted per the treating physician
Taking digoxin, a P-gp substrate with a narrow therapeutic window.
Severe renal impairment (GFR < 30 mL/min/1.73m2).
Uncontrolled infection.
Presence of any active clinically relevant medical condition that in the opinion of the treating physician would preclude patient from safely participating in the program.
Pregnant or breast-feeding or plan to become pregnant while on therapy.
12. IPD Sharing Statement
Citations:
PubMed Identifier
29710166
Citation
Gordon LB, Shappell H, Massaro J, D'Agostino RB Sr, Brazier J, Campbell SE, Kleinman ME, Kieran MW. Association of Lonafarnib Treatment vs No Treatment With Mortality Rate in Patients With Hutchinson-Gilford Progeria Syndrome. JAMA. 2018 Apr 24;319(16):1687-1695. doi: 10.1001/jama.2018.3264.
Results Reference
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Learn more about this trial
Lonafarnib for Patients With Hutchinson-Gilford Progeria Syndrome or Progeroid Laminopathy
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