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Long Term, Follow-on Study of Lomitapide in Patients With Homozygous Familial Hypercholesterolemia

Phase 3
Completed
Conditions
Familial Hypercholesterolemia
Interventions
Registration Number
NCT00943306
Lead Sponsor
Aegerion Pharmaceuticals, Inc.
Brief Summary

This was a long term follow on study to assess the continued long term safety and efficacy of lomitapide in patients with homozygous familial hypercholesterolemia.

Detailed Description

This was a phase III open label clinical trial to evaluate the long-term efficacy and safety of lomitapide at the maximum tolerated dose (for each patient) established during the clinical trial 733-005/UP1002. Subjects completing the 78-week treatment period in study 733-005/UP1002 who have not met any of the stopping criteria will be eligible to participate in 733-012. The treatment period continued until a decision was made by the local competent authority regarding marketing authorization. Lomitapide will be given orally once daily. Patient specific doses will be carried forward from 733-005/UP1002, but will not exceed the maximum tolerated dose the patient received during 733-005 /UP1002. The maximum dose for any patient was 80 mg/day. There was no reference therapy in this trial. The effects of the study drug were compared to baseline data (from 733-005/UP1002). Concomitant lipid-lowering therapy including plasmapheresis or LDL apheresis is permitted.

Note that hereafter, Week 0 in Study 733-005/UP1002 will be referred to as Baseline and Week 48 in Study AEGR-733-012 will be referred to as Week 126 (of overall treatment).

Recruitment & Eligibility

Status
COMPLETED
Sex
All
Target Recruitment
19
Inclusion Criteria
  1. Completed UP1002 or 733-005.
  2. Willing and able to provide consent and comply with the requirements of the study protocol.
Exclusion Criteria
  1. Met any of the stopping rules for study discontinuation at the final visit of study UP1002 or 733-005.

Study & Design

Study Type
INTERVENTIONAL
Study Design
SINGLE_GROUP
Arm && Interventions
GroupInterventionDescription
lomitapidelomitapideMaximum tolerated dose of lomitapide in addition to existing lipid lowering therapy including plasmapheresis or lipid apheresis.
Primary Outcome Measures
NameTimeMethod
Percent Change in Low Density Lipoprotein Cholesterol (LDL-C)Baseline and Week 126

Percent Change in Low Density Lipoprotein Cholesterol (LDL-C) from Baseline (Week 0 of Study 733-005/UP1002) to Week 126 (Week 48 in Study AEGR-733-012).

Secondary Outcome Measures
NameTimeMethod
Percent Change in Apolipoprotein B (Apo B)Baseline and Week 294

Percent change in Apolipoprotein B (Apo B) from Baseline (Week 0 of Study 733-005/UP1002) to Week 294 (Week 216 in Study AEGR-733-012).

Percent Change in Non High Density Lipoprotein Cholesterol (Non-HDL-C)Baseline and Week 294

Percent change in Non High Density Lipoprotein Cholesterol (Non-HDL-C) from Baseline (Week 0 of Study 733-005/UP1002) to Week 294 (Week 216 in Study AEGR-733-012).

Percent Change in Apolipoprotein AI (Apo AI)Baseline and Week 294

Percent change in Apolipoprotein AI (Apo AI) from Baseline (Week 0 of Study 733-005/UP1002) to Week 294 (Week 216 in Study AEGR-733-012).

Percent Change in Very Low Density Lipoprotein Cholesterol (VLDL-C)Baseline and Week 294

Percent change in Very Low Density Lipoprotein Cholesterol (VLDL-C) from Baseline (Week 0 of Study 733-005/UP1002) to Week 294 (Week 216 in Study AEGR-733-012).

Percent Change in Lp(a)Baseline and Week 294

Percent change in Lp(a) from Baseline (Week 0 of Study 733-005/UP1002) to Week 294 (Week 216 in Study AEGR-733-012).

Percent Change in TriglyceridesBaseline and Week 294

Percent change in Triglycerides from Baseline (Week 0 of Study 733-005/UP1002) to Week 294 (Week 216 in Study AEGR-733-012).

Percent Change in Low Density Lipoprotein Cholesterol (LDL-C)Baseline and Week 294

Percent change in Low Density Lipoprotein Cholesterol (LDL-C) from Baseline (Week 0 of Study 733-005/UP1002) to Week 294 (Week 216 in Study AEGR-733-012).

Percent Change in Total CholesterolBaseline and Week 294

Percent change in Total Cholesterol from Baseline (Week 0 of Study 733-005/UP1002) to Week 294 (Week 216 in Study AEGR-733-012).

Percent Change in High Density Lipoprotein Cholesterol (HDL-C)Baseline and Week 294

Percent change in High Density Lipoprotein Cholesterol (HDL-C) from Baseline (Week 0 of Study 733-005/UP1002) to Week 294 (Week 216 in Study AEGR-733-012).

Trial Locations

Locations (10)

Robarts Research Institute

🇨🇦

London, Ontario, Canada

Lipid Clinic and University of Montreal Community Genomic Medicine Center

🇨🇦

Chicoutimi, Quebec, Canada

University of Pennsylvania

🇺🇸

Philadelphia, Pennsylvania, United States

Cedars-Sinai Medical Center

🇺🇸

Los Angeles, California, United States

Dipartimento di Medicina Clinica e Delle Patologie Emergenti

🇮🇹

Palermo, Sicily, Italy

University of Capetown

🇿🇦

Cape town, South Africa

Medicina Interna Universitaria

🇮🇹

Ferrara, Sicily, Italy

Centro Universitario Dislipidemie

🇮🇹

Milano, Italy

DAI Ematologia, Oncologia, Anatomia Patologica e Medicina

🇮🇹

Roma, Italy

Cardiology Research

🇿🇦

Bloemfontein, South Africa

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