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Open Label Extension Study of 1 mg/kg Pegunigalsidase Alfa Every 2 Weeks in Patients With Fabry Disease

Phase 3
Completed
Conditions
Fabry Disease
Interventions
Registration Number
NCT03566017
Lead Sponsor
Chiesi Farmaceutici S.p.A.
Brief Summary

The objective of CLI-06657AA1-04 (formerly PB-102-F60) is to evaluate the long-term safety, tolerability, and efficacy parameters of 1 mg/kg pegunigalsidase alfa administered intravenously every other week in adult Fabry patients who have successfully completed studies PB-102-F03, PB-102-F20 or PB-102-F30.

Detailed Description

This is an open-label study. Patients will be enrolled to receive 1 mg/kg pegunigalsidase alfa as intravenous infusions every 2 weeks (±3 days). The duration of treatment is until pegunigalsidase alfa is commercially available to the patient, or at the discretion of the Sponsor. For the analysis, available efficacy and safety parameters will be summarized using descriptive statistics.

Recruitment & Eligibility

Status
COMPLETED
Sex
All
Target Recruitment
97
Inclusion Criteria
  1. Completion of study PB-102-F20, PB-102-F03, or PB-102-F30
  2. The patient signs informed consent
  3. Female patients and male patients whose co-partners are of child-bearing potential agree to use a medically acceptable method of contraception. These include combined (estrogen- and progestogen-containing) hormonal contraception associated with inhibition of ovulation (oral, intravaginal, or transdermal) supplemented with a barrier method (preferably male condom), progestogen-only hormonal contraception associated with inhibition of ovulation (oral, injectable, or implantable) supplemented with a barrier method (preferably male condom), intrauterine device (IUD), intrauterine hormone-releasing system (IUS), bilateral tubal occlusion, vasectomised partner, or sexual abstinence. Contraception should be used for 2 weeks after treatment termination.
Exclusion Criteria

Presence of any medical, emotional, behavioral or psychological condition that, in the judgment of the Investigator, would interfere with patient compliance with the requirements of the study.

Study & Design

Study Type
INTERVENTIONAL
Study Design
SINGLE_GROUP
Arm && Interventions
GroupInterventionDescription
Experimental open labelpegunigalsidase alfapegunigalsidase alfa
Primary Outcome Measures
NameTimeMethod
Evaluation of treatment-related adverse eventsThroughout the study, 7 years

CTCAE v4.03

Secondary Outcome Measures
NameTimeMethod
Kidney function 1Every 6 months throughout trial, 7 years

Estimated glomerular filtration rate (eGFRCKD-EPI)

Cardiac assessmentEvery 12 months to end of the study, 7 years

Left Ventricular Mass Index (g/m2) by magnetic resonance imaging (MRI) and Echocardiograph, and exercise tolerance (Stress Test)

Biomarkers for Fabry diseaseEvery 12 months to end of the study, 7 years. For patients from PB-102-F20 at 3 and 6 months.

plasma Lyso-Gb3 and Gb3

Record of pain medication useEvery two weeks for 7 years

Frequency of pain medication use, or pre-infusion medication

Kidney function 2Every 6 months to the end of the study, 7 years

Protein/Creatinine ratio, spot urine test (UPCR)

Pain assessmentEvery 6 months up to the end of the study, 7 years

short form Brief Pain Inventory (BPI)

Symptom assessmentEvery 12 months up to the end of the study, 7 years

Mainz Severity Score Index (MSSI)

Quality of life assessmentEvery 6 months up to the end of the study, 7 years

quality of life (EQ-5D-5L)

Trial Locations

Locations (30)

General Hospital Slovenj Gradec

🇸🇮

Slovenj Gradec, Slovenia

Infusion Associates

🇺🇸

Grand Rapids, Michigan, United States

Institute of Metabolic Disease

🇺🇸

Dallas, Texas, United States

University of Utah Hospitals & Clinics

🇺🇸

Salt Lake City, Utah, United States

UAB Medicine

🇺🇸

Birmingham, Alabama, United States

Phoenix Children's Hospital

🇺🇸

Phoenix, Arizona, United States

University of California San Diego

🇺🇸

La Jolla, California, United States

University of California Irvine Center

🇺🇸

Orange, California, United States

University of Florida

🇺🇸

Gainesville, Florida, United States

Emory University School of Medicine

🇺🇸

Atlanta, Georgia, United States

University of Iowa Hospitals and Clinics

🇺🇸

Iowa City, Iowa, United States

Cincinnati Children's Hospital Medical Center

🇺🇸

Cincinnati, Ohio, United States

University of Pittsburgh

🇺🇸

Pittsburgh, Pennsylvania, United States

Renal Disease Research Institute, LLC - Dallas

🇺🇸

Dallas, Texas, United States

O+O Alpan LLC

🇺🇸

Fairfax, Virginia, United States

Medical College of Wisconsin

🇺🇸

Milwaukee, Wisconsin, United States

Royal Melbourne Hospital

🇦🇺

Parkville, Victoria, Australia

Capital District Health Authority

🇨🇦

Halifax, Nova Scotia, Canada

Vseobecna fakultni nemocnice v Praze

🇨🇿

Prague, Czech Republic, Czechia

Turku University Central Hospital

🇫🇮

Turku, Finland

Hospital Raymond-Poincaré

🇫🇷

Garches, France

Semmelweis Egyetem

🇭🇺

Budapest, Hungary

Azienda Ospedaliera Universitaria "Federico II"

🇮🇹

Napoli, Via Pansini, Italy

Academisch Medisch Centrum

🇳🇱

Amsterdam, Netherlands

Haukeland University Hospital Klinisk Forskningspost

🇳🇴

Bergen, Norway

Hospital de Dia Quiron Zaragoza

🇪🇸

Zaragoza, Spain

University Hospitals Birmingham NHS Foundation Trust, Queen Elizabeth Hospital

🇬🇧

Edgbaston, Birmingham, United Kingdom

Salford Royal

🇬🇧

Salford, Greater Manchester, United Kingdom

Addenbrooke's Hospital

🇬🇧

Cambridge, United Kingdom

The Royal Free Hospital

🇬🇧

London, United Kingdom

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