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Dose Escalation Study of QR-010 in Homozygous ΔF508 Cystic Fibrosis Patients

Phase 1
Completed
Conditions
Cystic Fibrosis
Interventions
Drug: Placebo
Registration Number
NCT02532764
Lead Sponsor
ProQR Therapeutics
Brief Summary

A randomized, double-blind, placebo-controlled study of single and multiple ascending doses of QR-010 in adults homozygous for ΔF508 Cystic Fibrosis.

Detailed Description

The purpose of this study is to evaluate the safety, tolerability, and to determine the pharmacokinetics of QR-010 administered via inhalation in adult homozygous for ΔF508 Cystic Fibrosis.

Recruitment & Eligibility

Status
COMPLETED
Sex
All
Target Recruitment
70
Inclusion Criteria
  • Confirmed diagnosis of CF as defined by iontophoretic pilocarpine sweat chloride test (sweat chloride) of > 60 mmol/L
  • Confirmation of CFTR gene mutations homozygous for the ΔF508 mutation
  • Body mass index (BMI) ≥ 17 kg/m2
  • Non-smoking for a minimum of two years
  • FEV1 ≥70% of predicted normal for age, gender, and height, at Screening
  • Stable lung function
  • Adequate hepatic and renal function
Exclusion Criteria
  • Breast-feeding or pregnant
  • Use of lumacaftor or ivacaftor
  • Use of any investigational drug or device
  • History of lung transplantation
  • Hemoptysis

Study & Design

Study Type
INTERVENTIONAL
Study Design
SINGLE_GROUP
Arm && Interventions
GroupInterventionDescription
PlaceboPlaceboPlacebo (normal saline) administered via inhalation either as a single dose or three times weekly for four weeks.
QR-010QR-010QR-010 administered via inhalation either as a single dose or three times weekly for four weeks.
Primary Outcome Measures
NameTimeMethod
Incidence of Subjects Experiencing Dose-Limiting Toxicities (DLT) in Each Dose Cohort From Baseline Through End of Study Visit.8 Days for Single-dose cohorts; 8 weeks for Multiple-dose cohorts

DLT's were defined as an allergic reaction, acute bronchospasm or acute AEs of interest requiring (immediate) medical intervention.

Severity of Treatment Emergent Adverse Events From Baseline Through End of Study8 Days for Single-dose cohorts; 8 weeks for Multiple-dose cohorts

Assessment of severity of treatment emergent adverse events (TEAEs).

Severity is graded using the National Cancer Institute (NCI) Common Terminology Criteria for Adverse Events Modified for CF (CTCAE v4.03). For events not present in this listing the following grading was applied:

Mild: Asymptomatic or mild symptoms; clinical or diagnostic observations only; intervention not indicated; Moderate: Minimal, local, or noninvasive intervention indicated; discomfort sufficient to reduce or interfere with daily activities; Severe: Medically significant but not immediately life-threatening; hospitalization or prolongation of hospitalization may be indicated; disabling; limits self-care with significant interference with daily activities; incapacitating with inability to perform self care activities of daily living; Life-threatening: Urgent intervention indicated; immediate risk of death.

Incidence of Subjects Experiencing Treatment Emergent Adverse Events From Baseline Through End of Study8 Days for Single-dose cohorts; 8 weeks for Multiple-dose cohorts

Number of subjects experiencing at least one treatment emergent adverse events (TEAEs)

Secondary Outcome Measures
NameTimeMethod
Time to Maximum Serum Concentration8 Days for Single-dose cohorts; 8 weeks for Multiple-dose cohorts

Tmax: Time to Cmax of QR-010 serum concentrations.

Area Under the Curve to Infinity [AUC(0-∞)]8 Days for Single-dose cohorts; 8 weeks for Multiple-dose cohorts

AUC0-∞: Area under the curve to infinity will be calculated based on the last observed concentration Clast(obs) using formula: AUC0-∞=AUClast+Clast(obs)/λz

Number of Subjects With Abnormalities Reported Regarding Laboratory Parameters, Vital Signs, ECG, Spirometry, and Physical Findings.8 Days for Single-dose cohorts; 8 weeks for Multiple-dose cohorts

Number of subjects experiencing at least one abnormality for the categories laboratory parameters, vital signs, ECG, spirometry and physical findings that were reported as treatment emergent adverse event with a relationship to study drug as either possibly, probably or definitely.

Maximum Serum Concentration8 Days for Single-dose cohorts; 8 weeks for Multiple-dose cohorts

Cmax: QR-010 maximum serum concentrations

Terminal Half-life (T1/2)8 Days for Single-dose cohorts; 8 weeks for Multiple-dose cohorts

The terminal elimination half-life will be estimated by non-linear regression analysis of the terminal elimination slope

Area Under the Curve to Final Sample [AUC(0-last)]8 Days for Single-dose cohorts; 8 weeks for Multiple-dose cohorts

Area under the curve to the final sample with a concentration greater than lower limit of quantification (LLQ) will be calculated using the linear trapezoidal method

Serum Clearance (CL)8 Days for Single-dose cohorts; 8 weeks for Multiple-dose cohorts

CL: Serum clearance will be estimated using the formula: CL = Dose/AUC0-∞.

Trial Locations

Locations (27)

University of Southern California USC - Keck School of Medicine

🇺🇸

Los Angeles, California, United States

Boston Children's Hospital

🇺🇸

Boston, Massachusetts, United States

Northwestern University

🇺🇸

Chicago, Illinois, United States

Massachusetts General Hospital

🇺🇸

Boston, Massachusetts, United States

Nationwide Children's Hospital

🇺🇸

Columbus, Ohio, United States

Penn State Milton S. Hershey Medical Center

🇺🇸

Hershey, Pennsylvania, United States

University of Texas Southwestern Medical Center

🇺🇸

Dallas, Texas, United States

Motol University Hospital

🇨🇿

Prague, Czechia

University of Washington Medical Center

🇺🇸

Seattle, Washington, United States

Cystic Fibrosis Center Rigshospitalet

🇩🇰

Copenhagen, Denmark

Hopital Necker- Enfants Malades

🇫🇷

Paris, France

HGRL Chu Nantes

🇫🇷

Nantes, France

Charité Universitätsmedizin Berlin

🇩🇪

Berlin, Germany

Munich U. Hospital, Cystic Fibrosis Center for Adults

🇩🇪

Munich, Germany

Medizinische Hochschule Hannover

🇩🇪

Hannover, Germany

Hospital Vall D'Hebron

🇪🇸

Barcelona, Spain

Southampton General Hospital

🇬🇧

Southampton, United Kingdom

Medical University of South Carolina

🇺🇸

Charleston, South Carolina, United States

Washington University School of Medicine

🇺🇸

Saint Louis, Missouri, United States

Universitair Ziekenhuis Brussel

🇧🇪

Brussels, Belgium

University of Leuven

🇧🇪

Leuven, Belgium

Celerion

🇬🇧

Belfast, Northern Ireland, United Kingdom

University of Calgary (Health Sciences Centre)

🇨🇦

Calgary, Alberta, Canada

University of Kansas Medical Center Research Institute

🇺🇸

Kansas City, Kansas, United States

Azienda Ospedaliera Universitaria Integrata di Verona

🇮🇹

Verona, Italy

Stanford University

🇺🇸

Palo Alto, California, United States

Royal Brompton Hospital

🇬🇧

London, United Kingdom

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