Trial Outcomes & Findings for Safety of Fresolimumab in the Treatment of Osteogenesis Imperfecta (NCT NCT03064074)

NCT ID: NCT03064074

Last Updated: 2024-12-04

Results Overview

Safety of single and repeat doses of fresolimumab will be assessed in adult patients with moderate to severe osteogenesis imperfecta

Recruitment status

COMPLETED

Study phase

PHASE1

Target enrollment

11 participants

Primary outcome timeframe

6 months for single dose study and 12 months for repeat dose study

Results posted on

2024-12-04

Participant Flow

Participant milestones

Participant milestones
Measure
Stage 1 Low Dose
There are a total of 6 study visits within approximately a 6 month timespan. Investigators will evaluate the safety of a single administration of fresolimumab in adult patients with OI. Subjects will receive a single-dose of 1 mg/kg of fresolimumab (n=4). At each study visit, the participant may have the following testing done: * Physical exam * Vitals * Blood draw for safety labs, pharmacokinetics, etc * If the participant is female, she will have a pregnancy test * EKG * DXA * Infusion of the study drug Fresolimumab: The purpose of this study is to determine if fresolimumab is safe as a treatment for OI. We will evaluate the safety of a single dose of fresolimumab in the 1st stage of the study. We will evaluate the safety of multiple doses of fresolimumab in the 2nd stage of the study. The Investigators will evaluate the effect of the two doses of fresolimumab in Stage 1 on markers of bone turnover and determine the dose that shows the greatest reduction in bone turnover markers compared to no treatment. This dose will be chosen for the repeat dose studies. If there were no significant changes between the bone turnover markers with either dose, the 4 mg/kg dose will be chosen for the repeat dose study.
Stage 2 High Dose
There are a total of 6 study visits within approximately a 6 month timespan. Investigators will evaluate the safety of a single administration of fresolimumab in adult patients with OI. Subjects will receive a single-dose of 4 mg/kg of fresolimumab (n=4). At each study visit, the participant may have the following testing done: * Physical exam * Vitals * Blood draw for safety labs, pharmacokinetics, etc * If the participant is female, she will have a pregnancy test * EKG * DXA * Infusion of the study drug Fresolimumab: The purpose of this study is to determine if fresolimumab is safe as a treatment for OI. We will evaluate the safety of a single dose of fresolimumab in the 1st stage of the study. We will evaluate the safety of multiple doses of fresolimumab in the 2nd stage of the study. The Investigators will evaluate the effect of the two doses of fresolimumab in Stage 1 on markers of bone turnover and determine the dose that shows the greatest reduction in bone turnover markers compared to no treatment. This dose will be chosen for the repeat dose studies. If there were no significant changes between the bone turnover markers with either dose, the 4 mg/kg dose will be chosen for the repeat dose study.
Stage 2 Repeat Dose Every 6 Months
Fresolimumab will be administered every six months for a total treatment period of 12 months (n=4). The dose to be administered (1 or 4 mg/kg) will be chosen after completion of Stage 1. The primary Stage 2 endpoint will be safety measures assessed over 12 months. The secondary endpoints will be changes in markers of bone remodeling, bone mineral density, estimated strength. At each study visit, participants may have the following testing done: * Physical exam * Vitals * Blood draw for safety labs, pharmacokinetics, etc * If the participant is female, she will have a pregnancy test * EKG * DXA * Infusion of the study drug * Skeletal survey * Peripheral quantitative CT (pQCT) of the forearm * Quality of Life Surveys * Pulmonary function test * Walk test Fresolimumab: The purpose of this study is to determine if fresolimumab is safe as a treatment for OI. We will evaluate the safety of a single dose of fresolimumab in the 1st stage of the study. We will evaluate the safety of multiple doses of fresolimumab in the 2nd stage of the study. The Investigators will evaluate the effect of the two doses of fresolimumab in Stage 1 on markers of bone turnover and determine the dose that shows the greatest reduction in bone turnover markers compared to no treatment. This dose will be chosen for the repeat dose studies. If there were no significant changes between the bone turnover markers with either dose, the 4 mg/kg dose will be chosen for the repeat dose study.
Stage 2 Repeat Doses Every 3 Months
Fresolimumab will be administered every three months for a total treatment period of 12 months (n=4). The dose to be administered (1 or 4 mg/kg) will be chosen after completion of Stage 1. The primary Stage 2 endpoint will be safety measures assessed over 12 months. The secondary endpoints will be changes in markers of bone remodeling, bone mineral density, estimated strength. At each study visit, participants may have the following testing done: * Physical exam * Vitals * Blood draw for safety labs, pharmacokinetics, etc * If the participant is female, she will have a pregnancy test * EKG * DXA * Infusion of the study drug * Skeletal survey * Peripheral quantitative CT (pQCT) of the forearm * Quality of Life Surveys * Pulmonary function test * Walk test Fresolimumab: The purpose of this study is to determine if fresolimumab is safe as a treatment for OI. We will evaluate the safety of a single dose of fresolimumab in the 1st stage of the study. We will evaluate the safety of multiple doses of fresolimumab in the 2nd stage of the study. The Investigators will evaluate the effect of the two doses of fresolimumab in Stage 1 on markers of bone turnover and determine the dose that shows the greatest reduction in bone turnover markers compared to no treatment. This dose will be chosen for the repeat dose studies. If there were no significant changes between the bone turnover markers with either dose, the 4 mg/kg dose will be chosen for the repeat dose study.
Overall Study
STARTED
4
4
2
1
Overall Study
COMPLETED
4
4
2
0
Overall Study
NOT COMPLETED
0
0
0
1

Reasons for withdrawal

Reasons for withdrawal
Measure
Stage 1 Low Dose
There are a total of 6 study visits within approximately a 6 month timespan. Investigators will evaluate the safety of a single administration of fresolimumab in adult patients with OI. Subjects will receive a single-dose of 1 mg/kg of fresolimumab (n=4). At each study visit, the participant may have the following testing done: * Physical exam * Vitals * Blood draw for safety labs, pharmacokinetics, etc * If the participant is female, she will have a pregnancy test * EKG * DXA * Infusion of the study drug Fresolimumab: The purpose of this study is to determine if fresolimumab is safe as a treatment for OI. We will evaluate the safety of a single dose of fresolimumab in the 1st stage of the study. We will evaluate the safety of multiple doses of fresolimumab in the 2nd stage of the study. The Investigators will evaluate the effect of the two doses of fresolimumab in Stage 1 on markers of bone turnover and determine the dose that shows the greatest reduction in bone turnover markers compared to no treatment. This dose will be chosen for the repeat dose studies. If there were no significant changes between the bone turnover markers with either dose, the 4 mg/kg dose will be chosen for the repeat dose study.
Stage 2 High Dose
There are a total of 6 study visits within approximately a 6 month timespan. Investigators will evaluate the safety of a single administration of fresolimumab in adult patients with OI. Subjects will receive a single-dose of 4 mg/kg of fresolimumab (n=4). At each study visit, the participant may have the following testing done: * Physical exam * Vitals * Blood draw for safety labs, pharmacokinetics, etc * If the participant is female, she will have a pregnancy test * EKG * DXA * Infusion of the study drug Fresolimumab: The purpose of this study is to determine if fresolimumab is safe as a treatment for OI. We will evaluate the safety of a single dose of fresolimumab in the 1st stage of the study. We will evaluate the safety of multiple doses of fresolimumab in the 2nd stage of the study. The Investigators will evaluate the effect of the two doses of fresolimumab in Stage 1 on markers of bone turnover and determine the dose that shows the greatest reduction in bone turnover markers compared to no treatment. This dose will be chosen for the repeat dose studies. If there were no significant changes between the bone turnover markers with either dose, the 4 mg/kg dose will be chosen for the repeat dose study.
Stage 2 Repeat Dose Every 6 Months
Fresolimumab will be administered every six months for a total treatment period of 12 months (n=4). The dose to be administered (1 or 4 mg/kg) will be chosen after completion of Stage 1. The primary Stage 2 endpoint will be safety measures assessed over 12 months. The secondary endpoints will be changes in markers of bone remodeling, bone mineral density, estimated strength. At each study visit, participants may have the following testing done: * Physical exam * Vitals * Blood draw for safety labs, pharmacokinetics, etc * If the participant is female, she will have a pregnancy test * EKG * DXA * Infusion of the study drug * Skeletal survey * Peripheral quantitative CT (pQCT) of the forearm * Quality of Life Surveys * Pulmonary function test * Walk test Fresolimumab: The purpose of this study is to determine if fresolimumab is safe as a treatment for OI. We will evaluate the safety of a single dose of fresolimumab in the 1st stage of the study. We will evaluate the safety of multiple doses of fresolimumab in the 2nd stage of the study. The Investigators will evaluate the effect of the two doses of fresolimumab in Stage 1 on markers of bone turnover and determine the dose that shows the greatest reduction in bone turnover markers compared to no treatment. This dose will be chosen for the repeat dose studies. If there were no significant changes between the bone turnover markers with either dose, the 4 mg/kg dose will be chosen for the repeat dose study.
Stage 2 Repeat Doses Every 3 Months
Fresolimumab will be administered every three months for a total treatment period of 12 months (n=4). The dose to be administered (1 or 4 mg/kg) will be chosen after completion of Stage 1. The primary Stage 2 endpoint will be safety measures assessed over 12 months. The secondary endpoints will be changes in markers of bone remodeling, bone mineral density, estimated strength. At each study visit, participants may have the following testing done: * Physical exam * Vitals * Blood draw for safety labs, pharmacokinetics, etc * If the participant is female, she will have a pregnancy test * EKG * DXA * Infusion of the study drug * Skeletal survey * Peripheral quantitative CT (pQCT) of the forearm * Quality of Life Surveys * Pulmonary function test * Walk test Fresolimumab: The purpose of this study is to determine if fresolimumab is safe as a treatment for OI. We will evaluate the safety of a single dose of fresolimumab in the 1st stage of the study. We will evaluate the safety of multiple doses of fresolimumab in the 2nd stage of the study. The Investigators will evaluate the effect of the two doses of fresolimumab in Stage 1 on markers of bone turnover and determine the dose that shows the greatest reduction in bone turnover markers compared to no treatment. This dose will be chosen for the repeat dose studies. If there were no significant changes between the bone turnover markers with either dose, the 4 mg/kg dose will be chosen for the repeat dose study.
Overall Study
Physician Decision
0
0
0
1

Baseline Characteristics

Safety of Fresolimumab in the Treatment of Osteogenesis Imperfecta

Baseline characteristics by cohort

Baseline characteristics by cohort
Measure
Stage 1 Low Dose
n=4 Participants
There are a total of 6 study visits within approximately a 6 month timespan. Investigators will evaluate the safety of a single administration of fresolimumab in adult patients with OI. Subjects will receive a single-dose of 1 mg/kg of fresolimumab (n=4). At each study visit, the participant may have the following testing done: * Physical exam * Vitals * Blood draw for safety labs, pharmacokinetics, etc * If the participant is female, she will have a pregnancy test * EKG * DXA * Infusion of the study drug Fresolimumab: The purpose of this study is to determine if fresolimumab is safe as a treatment for OI. We will evaluate the safety of a single dose of fresolimumab in the 1st stage of the study. We will evaluate the safety of multiple doses of fresolimumab in the 2nd stage of the study. The Investigators will evaluate the effect of the two doses of fresolimumab in Stage 1 on markers of bone turnover and determine the dose that shows the greatest reduction in bone turnover markers compared to no treatment. This dose will be chosen for the repeat dose studies. If there were no significant changes between the bone turnover markers with either dose, the 4 mg/kg dose will be chosen for the repeat dose study.
Stage 2 High Dose
n=4 Participants
There are a total of 6 study visits within approximately a 6 month timespan. Investigators will evaluate the safety of a single administration of fresolimumab in adult patients with OI. Subjects will receive a single-dose of 4 mg/kg of fresolimumab (n=4). At each study visit, the participant may have the following testing done: * Physical exam * Vitals * Blood draw for safety labs, pharmacokinetics, etc * If the participant is female, she will have a pregnancy test * EKG * DXA * Infusion of the study drug Fresolimumab: The purpose of this study is to determine if fresolimumab is safe as a treatment for OI. We will evaluate the safety of a single dose of fresolimumab in the 1st stage of the study. We will evaluate the safety of multiple doses of fresolimumab in the 2nd stage of the study. The Investigators will evaluate the effect of the two doses of fresolimumab in Stage 1 on markers of bone turnover and determine the dose that shows the greatest reduction in bone turnover markers compared to no treatment. This dose will be chosen for the repeat dose studies. If there were no significant changes between the bone turnover markers with either dose, the 4 mg/kg dose will be chosen for the repeat dose study.
Stage 2 Repeat Dose Every 6 Months
n=2 Participants
Fresolimumab will be administered every six months for a total treatment period of 12 months (n=4). The dose to be administered (1 or 4 mg/kg) will be chosen after completion of Stage 1. The primary Stage 2 endpoint will be safety measures assessed over 12 months. The secondary endpoints will be changes in markers of bone remodeling, bone mineral density, estimated strength. At each study visit, participants may have the following testing done: * Physical exam * Vitals * Blood draw for safety labs, pharmacokinetics, etc * If the participant is female, she will have a pregnancy test * EKG * DXA * Infusion of the study drug * Skeletal survey * Peripheral quantitative CT (pQCT) of the forearm * Quality of Life Surveys * Pulmonary function test * Walk test Fresolimumab: The purpose of this study is to determine if fresolimumab is safe as a treatment for OI. We will evaluate the safety of a single dose of fresolimumab in the 1st stage of the study. We will evaluate the safety of multiple doses of fresolimumab in the 2nd stage of the study. The Investigators will evaluate the effect of the two doses of fresolimumab in Stage 1 on markers of bone turnover and determine the dose that shows the greatest reduction in bone turnover markers compared to no treatment. This dose will be chosen for the repeat dose studies. If there were no significant changes between the bone turnover markers with either dose, the 4 mg/kg dose will be chosen for the repeat dose study.
Stage 2 Repeat Doses Every 3 Months
n=1 Participants
Fresolimumab will be administered every three months for a total treatment period of 12 months (n=4). The dose to be administered (1 or 4 mg/kg) will be chosen after completion of Stage 1. The primary Stage 2 endpoint will be safety measures assessed over 12 months. The secondary endpoints will be changes in markers of bone remodeling, bone mineral density, estimated strength. At each study visit, participants may have the following testing done: * Physical exam * Vitals * Blood draw for safety labs, pharmacokinetics, etc * If the participant is female, she will have a pregnancy test * EKG * DXA * Infusion of the study drug * Skeletal survey * Peripheral quantitative CT (pQCT) of the forearm * Quality of Life Surveys * Pulmonary function test * Walk test Fresolimumab: The purpose of this study is to determine if fresolimumab is safe as a treatment for OI. We will evaluate the safety of a single dose of fresolimumab in the 1st stage of the study. We will evaluate the safety of multiple doses of fresolimumab in the 2nd stage of the study. The Investigators will evaluate the effect of the two doses of fresolimumab in Stage 1 on markers of bone turnover and determine the dose that shows the greatest reduction in bone turnover markers compared to no treatment. This dose will be chosen for the repeat dose studies. If there were no significant changes between the bone turnover markers with either dose, the 4 mg/kg dose will be chosen for the repeat dose study.
Total
n=11 Participants
Total of all reporting groups
Sex: Female, Male
Male
2 Participants
n=5 Participants
1 Participants
n=7 Participants
1 Participants
n=5 Participants
0 Participants
n=4 Participants
4 Participants
n=21 Participants
Age, Categorical
<=18 years
0 Participants
n=5 Participants
0 Participants
n=7 Participants
0 Participants
n=5 Participants
0 Participants
n=4 Participants
0 Participants
n=21 Participants
Age, Categorical
Between 18 and 65 years
4 Participants
n=5 Participants
4 Participants
n=7 Participants
2 Participants
n=5 Participants
1 Participants
n=4 Participants
11 Participants
n=21 Participants
Age, Categorical
>=65 years
0 Participants
n=5 Participants
0 Participants
n=7 Participants
0 Participants
n=5 Participants
0 Participants
n=4 Participants
0 Participants
n=21 Participants
Sex: Female, Male
Female
2 Participants
n=5 Participants
3 Participants
n=7 Participants
1 Participants
n=5 Participants
1 Participants
n=4 Participants
7 Participants
n=21 Participants
Race (NIH/OMB)
American Indian or Alaska Native
0 Participants
n=5 Participants
0 Participants
n=7 Participants
0 Participants
n=5 Participants
0 Participants
n=4 Participants
0 Participants
n=21 Participants
Race (NIH/OMB)
Asian
0 Participants
n=5 Participants
1 Participants
n=7 Participants
0 Participants
n=5 Participants
0 Participants
n=4 Participants
1 Participants
n=21 Participants
Race (NIH/OMB)
Native Hawaiian or Other Pacific Islander
0 Participants
n=5 Participants
0 Participants
n=7 Participants
0 Participants
n=5 Participants
0 Participants
n=4 Participants
0 Participants
n=21 Participants
Race (NIH/OMB)
Black or African American
0 Participants
n=5 Participants
0 Participants
n=7 Participants
0 Participants
n=5 Participants
0 Participants
n=4 Participants
0 Participants
n=21 Participants
Race (NIH/OMB)
White
3 Participants
n=5 Participants
2 Participants
n=7 Participants
2 Participants
n=5 Participants
1 Participants
n=4 Participants
8 Participants
n=21 Participants
Race (NIH/OMB)
More than one race
1 Participants
n=5 Participants
1 Participants
n=7 Participants
0 Participants
n=5 Participants
0 Participants
n=4 Participants
2 Participants
n=21 Participants
Race (NIH/OMB)
Unknown or Not Reported
0 Participants
n=5 Participants
0 Participants
n=7 Participants
0 Participants
n=5 Participants
0 Participants
n=4 Participants
0 Participants
n=21 Participants
Region of Enrollment
United States
4 participants
n=5 Participants
4 participants
n=7 Participants
2 participants
n=5 Participants
1 participants
n=4 Participants
11 participants
n=21 Participants

PRIMARY outcome

Timeframe: 6 months for single dose study and 12 months for repeat dose study

Safety of single and repeat doses of fresolimumab will be assessed in adult patients with moderate to severe osteogenesis imperfecta

Outcome measures

Outcome measures
Measure
Stage 1 Low Dose
n=4 Participants
There are a total of 6 study visits within approximately a 6 month timespan. Investigators will evaluate the safety of a single administration of fresolimumab in adult patients with OI. Subjects will receive a single-dose of 1 mg/kg of fresolimumab (n=4). At each study visit, the participant may have the following testing done: * Physical exam * Vitals * Blood draw for safety labs, pharmacokinetics, etc * If the participant is female, she will have a pregnancy test * EKG * DXA * Infusion of the study drug Fresolimumab: The purpose of this study is to determine if fresolimumab is safe as a treatment for OI. We will evaluate the safety of a single dose of fresolimumab in the 1st stage of the study. We will evaluate the safety of multiple doses of fresolimumab in the 2nd stage of the study. The Investigators will evaluate the effect of the two doses of fresolimumab in Stage 1 on markers of bone turnover and determine the dose that shows the greatest reduction in bone turnover markers compared to no treatment. This dose will be chosen for the repeat dose studies. If there were no significant changes between the bone turnover markers with either dose, the 4 mg/kg dose will be chosen for the repeat dose study.
Stage 2 High Dose
n=4 Participants
There are a total of 6 study visits within approximately a 6 month timespan. Investigators will evaluate the safety of a single administration of fresolimumab in adult patients with OI. Subjects will receive a single-dose of 4 mg/kg of fresolimumab (n=4). At each study visit, the participant may have the following testing done: * Physical exam * Vitals * Blood draw for safety labs, pharmacokinetics, etc * If the participant is female, she will have a pregnancy test * EKG * DXA * Infusion of the study drug Fresolimumab: The purpose of this study is to determine if fresolimumab is safe as a treatment for OI. We will evaluate the safety of a single dose of fresolimumab in the 1st stage of the study. We will evaluate the safety of multiple doses of fresolimumab in the 2nd stage of the study. The Investigators will evaluate the effect of the two doses of fresolimumab in Stage 1 on markers of bone turnover and determine the dose that shows the greatest reduction in bone turnover markers compared to no treatment. This dose will be chosen for the repeat dose studies. If there were no significant changes between the bone turnover markers with either dose, the 4 mg/kg dose will be chosen for the repeat dose study.
Stage 2 Repeat Dose Every 6 Months
n=2 Participants
Fresolimumab will be administered every six months for a total treatment period of 12 months (n=4). The dose to be administered (1 or 4 mg/kg) will be chosen after completion of Stage 1. The primary Stage 2 endpoint will be safety measures assessed over 12 months. The secondary endpoints will be changes in markers of bone remodeling, bone mineral density, estimated strength. At each study visit, participants may have the following testing done: * Physical exam * Vitals * Blood draw for safety labs, pharmacokinetics, etc * If the participant is female, she will have a pregnancy test * EKG * DXA * Infusion of the study drug * Skeletal survey * Peripheral quantitative CT (pQCT) of the forearm * Quality of Life Surveys * Pulmonary function test * Walk test Fresolimumab: The purpose of this study is to determine if fresolimumab is safe as a treatment for OI. We will evaluate the safety of a single dose of fresolimumab in the 1st stage of the study. We will evaluate the safety of multiple doses of fresolimumab in the 2nd stage of the study. The Investigators will evaluate the effect of the two doses of fresolimumab in Stage 1 on markers of bone turnover and determine the dose that shows the greatest reduction in bone turnover markers compared to no treatment. This dose will be chosen for the repeat dose studies. If there were no significant changes between the bone turnover markers with either dose, the 4 mg/kg dose will be chosen for the repeat dose study.
Stage 2 Repeat Doses Every 3 Months
n=1 Participants
Fresolimumab will be administered every three months for a total treatment period of 12 months (n=4). The dose to be administered (1 or 4 mg/kg) will be chosen after completion of Stage 1. The primary Stage 2 endpoint will be safety measures assessed over 12 months. The secondary endpoints will be changes in markers of bone remodeling, bone mineral density, estimated strength. At each study visit, participants may have the following testing done: * Physical exam * Vitals * Blood draw for safety labs, pharmacokinetics, etc * If the participant is female, she will have a pregnancy test * EKG * DXA * Infusion of the study drug * Skeletal survey * Peripheral quantitative CT (pQCT) of the forearm * Quality of Life Surveys * Pulmonary function test * Walk test Fresolimumab: The purpose of this study is to determine if fresolimumab is safe as a treatment for OI. We will evaluate the safety of a single dose of fresolimumab in the 1st stage of the study. We will evaluate the safety of multiple doses of fresolimumab in the 2nd stage of the study. The Investigators will evaluate the effect of the two doses of fresolimumab in Stage 1 on markers of bone turnover and determine the dose that shows the greatest reduction in bone turnover markers compared to no treatment. This dose will be chosen for the repeat dose studies. If there were no significant changes between the bone turnover markers with either dose, the 4 mg/kg dose will be chosen for the repeat dose study.
Number of Participants With Treatment-related Adverse Events as Assessed by CTCAE v4.0
2 Participants
4 Participants
2 Participants
1 Participants

SECONDARY outcome

Timeframe: 6 months in single dose study and 12 months in repeat dose study

Percentage change in bone turnover markers from baseline to Day 180

Outcome measures

Outcome measures
Measure
Stage 1 Low Dose
n=4 Participants
There are a total of 6 study visits within approximately a 6 month timespan. Investigators will evaluate the safety of a single administration of fresolimumab in adult patients with OI. Subjects will receive a single-dose of 1 mg/kg of fresolimumab (n=4). At each study visit, the participant may have the following testing done: * Physical exam * Vitals * Blood draw for safety labs, pharmacokinetics, etc * If the participant is female, she will have a pregnancy test * EKG * DXA * Infusion of the study drug Fresolimumab: The purpose of this study is to determine if fresolimumab is safe as a treatment for OI. We will evaluate the safety of a single dose of fresolimumab in the 1st stage of the study. We will evaluate the safety of multiple doses of fresolimumab in the 2nd stage of the study. The Investigators will evaluate the effect of the two doses of fresolimumab in Stage 1 on markers of bone turnover and determine the dose that shows the greatest reduction in bone turnover markers compared to no treatment. This dose will be chosen for the repeat dose studies. If there were no significant changes between the bone turnover markers with either dose, the 4 mg/kg dose will be chosen for the repeat dose study.
Stage 2 High Dose
n=4 Participants
There are a total of 6 study visits within approximately a 6 month timespan. Investigators will evaluate the safety of a single administration of fresolimumab in adult patients with OI. Subjects will receive a single-dose of 4 mg/kg of fresolimumab (n=4). At each study visit, the participant may have the following testing done: * Physical exam * Vitals * Blood draw for safety labs, pharmacokinetics, etc * If the participant is female, she will have a pregnancy test * EKG * DXA * Infusion of the study drug Fresolimumab: The purpose of this study is to determine if fresolimumab is safe as a treatment for OI. We will evaluate the safety of a single dose of fresolimumab in the 1st stage of the study. We will evaluate the safety of multiple doses of fresolimumab in the 2nd stage of the study. The Investigators will evaluate the effect of the two doses of fresolimumab in Stage 1 on markers of bone turnover and determine the dose that shows the greatest reduction in bone turnover markers compared to no treatment. This dose will be chosen for the repeat dose studies. If there were no significant changes between the bone turnover markers with either dose, the 4 mg/kg dose will be chosen for the repeat dose study.
Stage 2 Repeat Dose Every 6 Months
n=2 Participants
Fresolimumab will be administered every six months for a total treatment period of 12 months (n=4). The dose to be administered (1 or 4 mg/kg) will be chosen after completion of Stage 1. The primary Stage 2 endpoint will be safety measures assessed over 12 months. The secondary endpoints will be changes in markers of bone remodeling, bone mineral density, estimated strength. At each study visit, participants may have the following testing done: * Physical exam * Vitals * Blood draw for safety labs, pharmacokinetics, etc * If the participant is female, she will have a pregnancy test * EKG * DXA * Infusion of the study drug * Skeletal survey * Peripheral quantitative CT (pQCT) of the forearm * Quality of Life Surveys * Pulmonary function test * Walk test Fresolimumab: The purpose of this study is to determine if fresolimumab is safe as a treatment for OI. We will evaluate the safety of a single dose of fresolimumab in the 1st stage of the study. We will evaluate the safety of multiple doses of fresolimumab in the 2nd stage of the study. The Investigators will evaluate the effect of the two doses of fresolimumab in Stage 1 on markers of bone turnover and determine the dose that shows the greatest reduction in bone turnover markers compared to no treatment. This dose will be chosen for the repeat dose studies. If there were no significant changes between the bone turnover markers with either dose, the 4 mg/kg dose will be chosen for the repeat dose study.
Stage 2 Repeat Doses Every 3 Months
n=1 Participants
Fresolimumab will be administered every three months for a total treatment period of 12 months (n=4). The dose to be administered (1 or 4 mg/kg) will be chosen after completion of Stage 1. The primary Stage 2 endpoint will be safety measures assessed over 12 months. The secondary endpoints will be changes in markers of bone remodeling, bone mineral density, estimated strength. At each study visit, participants may have the following testing done: * Physical exam * Vitals * Blood draw for safety labs, pharmacokinetics, etc * If the participant is female, she will have a pregnancy test * EKG * DXA * Infusion of the study drug * Skeletal survey * Peripheral quantitative CT (pQCT) of the forearm * Quality of Life Surveys * Pulmonary function test * Walk test Fresolimumab: The purpose of this study is to determine if fresolimumab is safe as a treatment for OI. We will evaluate the safety of a single dose of fresolimumab in the 1st stage of the study. We will evaluate the safety of multiple doses of fresolimumab in the 2nd stage of the study. The Investigators will evaluate the effect of the two doses of fresolimumab in Stage 1 on markers of bone turnover and determine the dose that shows the greatest reduction in bone turnover markers compared to no treatment. This dose will be chosen for the repeat dose studies. If there were no significant changes between the bone turnover markers with either dose, the 4 mg/kg dose will be chosen for the repeat dose study.
Percentage Change in Bone Turnover Markers or P1NP, Osteocalcin or Ocn, and C-terminal Telopeptide or CTX
Percentage Change in CTX
49.89 Percent change
Standard Error 29.29
-4.02 Percent change
Standard Error 33.82
51.83 Percent change
Standard Error 34.56
NA Percent change
Standard Error NA
Only one participant was enrolled. This participant had to be withdrawn due to fracture because of OI and could not complete the study procedures. Thus, the percentage change of CTX could not be calculated.
Percentage Change in Bone Turnover Markers or P1NP, Osteocalcin or Ocn, and C-terminal Telopeptide or CTX
Percentage change in Osteocalcin
76.4 Percent change
Standard Error 39.1
-18.6 Percent change
Standard Error 39.13
83.9 Percent change
Standard Error 17.56
NA Percent change
Standard Error NA
Only one participant was enrolled. This participant had to be withdrawn due to fracture because of OI and could not complete the study procedures. Thus, the percentage change in Ocn could not be calculated.
Percentage Change in Bone Turnover Markers or P1NP, Osteocalcin or Ocn, and C-terminal Telopeptide or CTX
Percentage Change in P1NP
28.31 Percent change
Standard Error 17.44
24.95 Percent change
Standard Error 17.44
118.63 Percent change
Standard Error 45.40
NA Percent change
Standard Error NA
Only one participant was enrolled. This participant had to be withdrawn due to fracture because of OI and could not complete the study procedures. Thus, the percentage change of P1NP could not be calculated.

Adverse Events

Stage 1 Low Dose

Serious events: 0 serious events
Other events: 4 other events
Deaths: 0 deaths

Stage 2 High Dose

Serious events: 0 serious events
Other events: 4 other events
Deaths: 0 deaths

Stage 2 Repeat Dose Every 6 Months

Serious events: 1 serious events
Other events: 2 other events
Deaths: 0 deaths

Stage 2 Repeat Doses Every 3 Months

Serious events: 1 serious events
Other events: 1 other events
Deaths: 0 deaths

Serious adverse events

Serious adverse events
Measure
Stage 1 Low Dose
n=4 participants at risk
There are a total of 6 study visits within approximately a 6 month timespan. Investigators will evaluate the safety of a single administration of fresolimumab in adult patients with OI. Subjects will receive a single-dose of 1 mg/kg of fresolimumab (n=4). At each study visit, the participant may have the following testing done: * Physical exam * Vitals * Blood draw for safety labs, pharmacokinetics, etc * If the participant is female, she will have a pregnancy test * EKG * DXA * Infusion of the study drug Fresolimumab: The purpose of this study is to determine if fresolimumab is safe as a treatment for OI. We will evaluate the safety of a single dose of fresolimumab in the 1st stage of the study. We will evaluate the safety of multiple doses of fresolimumab in the 2nd stage of the study. The Investigators will evaluate the effect of the two doses of fresolimumab in Stage 1 on markers of bone turnover and determine the dose that shows the greatest reduction in bone turnover markers compared to no treatment. This dose will be chosen for the repeat dose studies. If there were no significant changes between the bone turnover markers with either dose, the 4 mg/kg dose will be chosen for the repeat dose study.
Stage 2 High Dose
n=4 participants at risk
There are a total of 6 study visits within approximately a 6 month timespan. Investigators will evaluate the safety of a single administration of fresolimumab in adult patients with OI. Subjects will receive a single-dose of 4 mg/kg of fresolimumab (n=4). At each study visit, the participant may have the following testing done: * Physical exam * Vitals * Blood draw for safety labs, pharmacokinetics, etc * If the participant is female, she will have a pregnancy test * EKG * DXA * Infusion of the study drug Fresolimumab: The purpose of this study is to determine if fresolimumab is safe as a treatment for OI. We will evaluate the safety of a single dose of fresolimumab in the 1st stage of the study. We will evaluate the safety of multiple doses of fresolimumab in the 2nd stage of the study. The Investigators will evaluate the effect of the two doses of fresolimumab in Stage 1 on markers of bone turnover and determine the dose that shows the greatest reduction in bone turnover markers compared to no treatment. This dose will be chosen for the repeat dose studies. If there were no significant changes between the bone turnover markers with either dose, the 4 mg/kg dose will be chosen for the repeat dose study.
Stage 2 Repeat Dose Every 6 Months
n=2 participants at risk
Fresolimumab will be administered every six months for a total treatment period of 12 months (n=4). The dose to be administered (1 or 4 mg/kg) will be chosen after completion of Stage 1. The primary Stage 2 endpoint will be safety measures assessed over 12 months. The secondary endpoints will be changes in markers of bone remodeling, bone mineral density, estimated strength. At each study visit, participants may have the following testing done: * Physical exam * Vitals * Blood draw for safety labs, pharmacokinetics, etc * If the participant is female, she will have a pregnancy test * EKG * DXA * Infusion of the study drug * Skeletal survey * Peripheral quantitative CT (pQCT) of the forearm * Quality of Life Surveys * Pulmonary function test * Walk test Fresolimumab: The purpose of this study is to determine if fresolimumab is safe as a treatment for OI. We will evaluate the safety of a single dose of fresolimumab in the 1st stage of the study. We will evaluate the safety of multiple doses of fresolimumab in the 2nd stage of the study. The Investigators will evaluate the effect of the two doses of fresolimumab in Stage 1 on markers of bone turnover and determine the dose that shows the greatest reduction in bone turnover markers compared to no treatment. This dose will be chosen for the repeat dose studies. If there were no significant changes between the bone turnover markers with either dose, the 4 mg/kg dose will be chosen for the repeat dose study.
Stage 2 Repeat Doses Every 3 Months
n=1 participants at risk
Fresolimumab will be administered every three months for a total treatment period of 12 months (n=4). The dose to be administered (1 or 4 mg/kg) will be chosen after completion of Stage 1. The primary Stage 2 endpoint will be safety measures assessed over 12 months. The secondary endpoints will be changes in markers of bone remodeling, bone mineral density, estimated strength. At each study visit, participants may have the following testing done: * Physical exam * Vitals * Blood draw for safety labs, pharmacokinetics, etc * If the participant is female, she will have a pregnancy test * EKG * DXA * Infusion of the study drug * Skeletal survey * Peripheral quantitative CT (pQCT) of the forearm * Quality of Life Surveys * Pulmonary function test * Walk test Fresolimumab: The purpose of this study is to determine if fresolimumab is safe as a treatment for OI. We will evaluate the safety of a single dose of fresolimumab in the 1st stage of the study. We will evaluate the safety of multiple doses of fresolimumab in the 2nd stage of the study. The Investigators will evaluate the effect of the two doses of fresolimumab in Stage 1 on markers of bone turnover and determine the dose that shows the greatest reduction in bone turnover markers compared to no treatment. This dose will be chosen for the repeat dose studies. If there were no significant changes between the bone turnover markers with either dose, the 4 mg/kg dose will be chosen for the repeat dose study.
Musculoskeletal and connective tissue disorders
Fracture
0.00%
0/4 • 6 Months for single dose study and 1 year for the multi dose study
0.00%
0/4 • 6 Months for single dose study and 1 year for the multi dose study
50.0%
1/2 • Number of events 1 • 6 Months for single dose study and 1 year for the multi dose study
100.0%
1/1 • Number of events 2 • 6 Months for single dose study and 1 year for the multi dose study

Other adverse events

Other adverse events
Measure
Stage 1 Low Dose
n=4 participants at risk
There are a total of 6 study visits within approximately a 6 month timespan. Investigators will evaluate the safety of a single administration of fresolimumab in adult patients with OI. Subjects will receive a single-dose of 1 mg/kg of fresolimumab (n=4). At each study visit, the participant may have the following testing done: * Physical exam * Vitals * Blood draw for safety labs, pharmacokinetics, etc * If the participant is female, she will have a pregnancy test * EKG * DXA * Infusion of the study drug Fresolimumab: The purpose of this study is to determine if fresolimumab is safe as a treatment for OI. We will evaluate the safety of a single dose of fresolimumab in the 1st stage of the study. We will evaluate the safety of multiple doses of fresolimumab in the 2nd stage of the study. The Investigators will evaluate the effect of the two doses of fresolimumab in Stage 1 on markers of bone turnover and determine the dose that shows the greatest reduction in bone turnover markers compared to no treatment. This dose will be chosen for the repeat dose studies. If there were no significant changes between the bone turnover markers with either dose, the 4 mg/kg dose will be chosen for the repeat dose study.
Stage 2 High Dose
n=4 participants at risk
There are a total of 6 study visits within approximately a 6 month timespan. Investigators will evaluate the safety of a single administration of fresolimumab in adult patients with OI. Subjects will receive a single-dose of 4 mg/kg of fresolimumab (n=4). At each study visit, the participant may have the following testing done: * Physical exam * Vitals * Blood draw for safety labs, pharmacokinetics, etc * If the participant is female, she will have a pregnancy test * EKG * DXA * Infusion of the study drug Fresolimumab: The purpose of this study is to determine if fresolimumab is safe as a treatment for OI. We will evaluate the safety of a single dose of fresolimumab in the 1st stage of the study. We will evaluate the safety of multiple doses of fresolimumab in the 2nd stage of the study. The Investigators will evaluate the effect of the two doses of fresolimumab in Stage 1 on markers of bone turnover and determine the dose that shows the greatest reduction in bone turnover markers compared to no treatment. This dose will be chosen for the repeat dose studies. If there were no significant changes between the bone turnover markers with either dose, the 4 mg/kg dose will be chosen for the repeat dose study.
Stage 2 Repeat Dose Every 6 Months
n=2 participants at risk
Fresolimumab will be administered every six months for a total treatment period of 12 months (n=4). The dose to be administered (1 or 4 mg/kg) will be chosen after completion of Stage 1. The primary Stage 2 endpoint will be safety measures assessed over 12 months. The secondary endpoints will be changes in markers of bone remodeling, bone mineral density, estimated strength. At each study visit, participants may have the following testing done: * Physical exam * Vitals * Blood draw for safety labs, pharmacokinetics, etc * If the participant is female, she will have a pregnancy test * EKG * DXA * Infusion of the study drug * Skeletal survey * Peripheral quantitative CT (pQCT) of the forearm * Quality of Life Surveys * Pulmonary function test * Walk test Fresolimumab: The purpose of this study is to determine if fresolimumab is safe as a treatment for OI. We will evaluate the safety of a single dose of fresolimumab in the 1st stage of the study. We will evaluate the safety of multiple doses of fresolimumab in the 2nd stage of the study. The Investigators will evaluate the effect of the two doses of fresolimumab in Stage 1 on markers of bone turnover and determine the dose that shows the greatest reduction in bone turnover markers compared to no treatment. This dose will be chosen for the repeat dose studies. If there were no significant changes between the bone turnover markers with either dose, the 4 mg/kg dose will be chosen for the repeat dose study.
Stage 2 Repeat Doses Every 3 Months
n=1 participants at risk
Fresolimumab will be administered every three months for a total treatment period of 12 months (n=4). The dose to be administered (1 or 4 mg/kg) will be chosen after completion of Stage 1. The primary Stage 2 endpoint will be safety measures assessed over 12 months. The secondary endpoints will be changes in markers of bone remodeling, bone mineral density, estimated strength. At each study visit, participants may have the following testing done: * Physical exam * Vitals * Blood draw for safety labs, pharmacokinetics, etc * If the participant is female, she will have a pregnancy test * EKG * DXA * Infusion of the study drug * Skeletal survey * Peripheral quantitative CT (pQCT) of the forearm * Quality of Life Surveys * Pulmonary function test * Walk test Fresolimumab: The purpose of this study is to determine if fresolimumab is safe as a treatment for OI. We will evaluate the safety of a single dose of fresolimumab in the 1st stage of the study. We will evaluate the safety of multiple doses of fresolimumab in the 2nd stage of the study. The Investigators will evaluate the effect of the two doses of fresolimumab in Stage 1 on markers of bone turnover and determine the dose that shows the greatest reduction in bone turnover markers compared to no treatment. This dose will be chosen for the repeat dose studies. If there were no significant changes between the bone turnover markers with either dose, the 4 mg/kg dose will be chosen for the repeat dose study.
Investigations
General Adverse events
100.0%
4/4 • Number of events 38 • 6 Months for single dose study and 1 year for the multi dose study
100.0%
4/4 • Number of events 24 • 6 Months for single dose study and 1 year for the multi dose study
100.0%
2/2 • Number of events 13 • 6 Months for single dose study and 1 year for the multi dose study
100.0%
1/1 • Number of events 21 • 6 Months for single dose study and 1 year for the multi dose study

Additional Information

dianne Nguyen

Baylor

Phone: 7137986694

Results disclosure agreements

  • Principal investigator is a sponsor employee
  • Publication restrictions are in place