Trial Outcomes & Findings for Safety of a Single Intravenous Dose of Recombinant Factor XIII in Children With Congenital FXIII A-subunit Deficiency (NCT NCT01230021)

NCT ID: NCT01230021

Last Updated: 2017-02-24

Results Overview

A measure of the exposure. Blood samples for the PK assessment were drawn pre-dose and up to 30 days after dosing. The PK of FXIII in children was assessed after a single i.v. dose of rFXIII 35 IU/kg.

Recruitment status

COMPLETED

Study phase

PHASE3

Target enrollment

6 participants

Primary outcome timeframe

At pre-dose, 30 minutes, 24 hours, 7, 14, 21 and 30 days after dosing

Results posted on

2017-02-24

Participant Flow

The trial was conducted at five sites located in the UK (2 sites), Israel (1 site) and the US (2 sites).

Between screening and treatment with trial drug the children were assessed for eligibility. If eligible, the children were treated with one single dose of FXIII. The trial was not randomised.

Participant milestones

Participant milestones
Measure
Recombinant Factor XIII
One single dose of 35 IU/kg rFXIII was administered as an intravenous (i.v.) injection to each child. The trial included one screening visit, one treatment visit (including pharmacokinetics (PK) assessments up to 24 hours) and 4 follow-up visits (at 7, 14, 21 and 30 days after dosing). Blood samples for PK assessments were drawn pre-dose and up to 30 days after dosing.
Overall Study
STARTED
6
Overall Study
COMPLETED
6
Overall Study
NOT COMPLETED
0

Reasons for withdrawal

Withdrawal data not reported

Baseline Characteristics

Safety of a Single Intravenous Dose of Recombinant Factor XIII in Children With Congenital FXIII A-subunit Deficiency

Baseline characteristics by cohort

Baseline characteristics by cohort
Measure
Recombinant Factor XIII
n=6 Participants
One single dose of 35 IU/kg rFXIII was administered as an intravenous (i.v.) injection to each child. The trial included one screening visit, one treatment visit (including pharmacokinetics (PK) assessments up to 24 hours) and 4 follow-up visits (at 7, 14, 21 and 30 days after dosing). Blood samples for PK assessments were drawn pre-dose and up to 30 days after dosing.
Age, Continuous
2.67 years
STANDARD_DEVIATION 1.03 • n=39 Participants
Gender
Female
3 Participants
n=39 Participants
Gender
Male
3 Participants
n=39 Participants
Ethnicity (NIH/OMB)
Hispanic or Latino
0 Participants
n=39 Participants
Ethnicity (NIH/OMB)
Not Hispanic or Latino
6 Participants
n=39 Participants
Ethnicity (NIH/OMB)
Unknown or Not Reported
0 Participants
n=39 Participants
Race (NIH/OMB)
American Indian or Alaska Native
0 Participants
n=39 Participants
Race (NIH/OMB)
Asian
3 Participants
n=39 Participants
Race (NIH/OMB)
Native Hawaiian or Other Pacific Islander
0 Participants
n=39 Participants
Race (NIH/OMB)
Black or African American
1 Participants
n=39 Participants
Race (NIH/OMB)
White
2 Participants
n=39 Participants
Race (NIH/OMB)
More than one race
0 Participants
n=39 Participants
Race (NIH/OMB)
Unknown or Not Reported
0 Participants
n=39 Participants

PRIMARY outcome

Timeframe: At pre-dose, 30 minutes, 24 hours, 7, 14, 21 and 30 days after dosing

Population: The full analysis set including all 6 children exposed to FXIII. All 6 children were exposed to one dose of trial product.

A measure of the exposure. Blood samples for the PK assessment were drawn pre-dose and up to 30 days after dosing. The PK of FXIII in children was assessed after a single i.v. dose of rFXIII 35 IU/kg.

Outcome measures

Outcome measures
Measure
Recombinant Factor XIII
n=6 Participants
One single dose of 35 IU/kg rFXIII was administered as an intravenous (i.v.) injection to each child. The trial included one screening visit, one treatment visit (including pharmacokinetics (PK) assessments up to 24 hours) and 4 follow-up visits (at 7, 14, 21 and 30 days after dosing). Blood samples for PK assessments were drawn pre-dose and up to 30 days after dosing.
Area Under the Concentration vs. Time Curve (AUC)
250.25 IU*h/mL
Standard Deviation 31.19

SECONDARY outcome

Timeframe: From day 0 to day 30

Population: The full analysis set including all 6 children exposed to FXIII. All 6 children were exposed to one dose of trial product.

A measure of exposure.

Outcome measures

Outcome measures
Measure
Recombinant Factor XIII
n=6 Participants
One single dose of 35 IU/kg rFXIII was administered as an intravenous (i.v.) injection to each child. The trial included one screening visit, one treatment visit (including pharmacokinetics (PK) assessments up to 24 hours) and 4 follow-up visits (at 7, 14, 21 and 30 days after dosing). Blood samples for PK assessments were drawn pre-dose and up to 30 days after dosing.
Area Under the Concentration vs. Time Curve (AUC0-∞)
403.18 IU*h/mL
Standard Deviation 92.20

SECONDARY outcome

Timeframe: At pre-dose, 30 minutes, 24 hours, 7, 14, 21 and 30 days after dosing

Population: The full analysis set including all 6 children exposed to FXIII. All 6 children were exposed to one dose of trial product.

Maximum plasma concentration of the drug reached.

Outcome measures

Outcome measures
Measure
Recombinant Factor XIII
n=6 Participants
One single dose of 35 IU/kg rFXIII was administered as an intravenous (i.v.) injection to each child. The trial included one screening visit, one treatment visit (including pharmacokinetics (PK) assessments up to 24 hours) and 4 follow-up visits (at 7, 14, 21 and 30 days after dosing). Blood samples for PK assessments were drawn pre-dose and up to 30 days after dosing.
Maximum Plasma Concentration (Cmax) for FXIII
0.69 U/mL
Standard Deviation 0.14

SECONDARY outcome

Timeframe: From day 0 to day 30

Population: The full analysis set including all 6 children exposed to FXIII. All 6 children were exposed to one dose of trial product.

Time point when half of the maximum plasma concentration is reached.

Outcome measures

Outcome measures
Measure
Recombinant Factor XIII
n=6 Participants
One single dose of 35 IU/kg rFXIII was administered as an intravenous (i.v.) injection to each child. The trial included one screening visit, one treatment visit (including pharmacokinetics (PK) assessments up to 24 hours) and 4 follow-up visits (at 7, 14, 21 and 30 days after dosing). Blood samples for PK assessments were drawn pre-dose and up to 30 days after dosing.
Terminal Half-life (t½)
378 hours
Interval 236.0 to 596.0

SECONDARY outcome

Timeframe: From day 0 to day 30

Population: The full analysis set including all 6 children exposed to FXIII. All 6 children were exposed to one dose of trial product.

The mean residence time (MRT) of a drug in the body and related functions are derived for drugs which are intravenously administered.

Outcome measures

Outcome measures
Measure
Recombinant Factor XIII
n=6 Participants
One single dose of 35 IU/kg rFXIII was administered as an intravenous (i.v.) injection to each child. The trial included one screening visit, one treatment visit (including pharmacokinetics (PK) assessments up to 24 hours) and 4 follow-up visits (at 7, 14, 21 and 30 days after dosing). Blood samples for PK assessments were drawn pre-dose and up to 30 days after dosing.
Mean Residence Time (MRT)
729.01 hours
Standard Deviation 265.98

SECONDARY outcome

Timeframe: From day 0 to day 30

Population: The full analysis set including all 6 children exposed to FXIII. All 6 children were exposed to one dose of trial product.

The total plasma clearance is a measure of the elimination of a drug from the body. Drugs are excreted primarily by the kidneys into the urine. Clearance is calculated as 'CL=Dose / AUC0-30 days').

Outcome measures

Outcome measures
Measure
Recombinant Factor XIII
n=6 Participants
One single dose of 35 IU/kg rFXIII was administered as an intravenous (i.v.) injection to each child. The trial included one screening visit, one treatment visit (including pharmacokinetics (PK) assessments up to 24 hours) and 4 follow-up visits (at 7, 14, 21 and 30 days after dosing). Blood samples for PK assessments were drawn pre-dose and up to 30 days after dosing.
Total Plasma Clearance (CL)
0.10 mL/h/kg
Standard Deviation 0.02

SECONDARY outcome

Timeframe: At steady state

Population: The full analysis set including all 6 children exposed to FXIII. All 6 children were exposed to one dose of trial product.

Volume of distribution at steady state (Vss) is the theoretical volume that the total amount of administered drug would have to occupy (if it were uniformly distributed), to provide the same concentration as it is in blood plasma at steady state. Steady state is achieved when all variables are constant in spite of ongoing processes.

Outcome measures

Outcome measures
Measure
Recombinant Factor XIII
n=6 Participants
One single dose of 35 IU/kg rFXIII was administered as an intravenous (i.v.) injection to each child. The trial included one screening visit, one treatment visit (including pharmacokinetics (PK) assessments up to 24 hours) and 4 follow-up visits (at 7, 14, 21 and 30 days after dosing). Blood samples for PK assessments were drawn pre-dose and up to 30 days after dosing.
Volume of Distribution at Steady State (Vss)
66.07 mL/kg
Standard Deviation 14.32

SECONDARY outcome

Timeframe: From day 0 to day 30

Population: Safety analysis population including all 6 children exposed to FXIII. All 6 children were exposed to one dose of trial product.

Outcome measures

Outcome measures
Measure
Recombinant Factor XIII
n=6 Participants
One single dose of 35 IU/kg rFXIII was administered as an intravenous (i.v.) injection to each child. The trial included one screening visit, one treatment visit (including pharmacokinetics (PK) assessments up to 24 hours) and 4 follow-up visits (at 7, 14, 21 and 30 days after dosing). Blood samples for PK assessments were drawn pre-dose and up to 30 days after dosing.
Percentage of Subjects With One or More Adverse Events (AEs) Recorded
33.3 percentage (%) of subjects

SECONDARY outcome

Timeframe: From day 0 to day 30

Population: Safety analysis population including all 6 children exposed to FXIII. All 6 children were exposed to one dose of trial product.

Outcome measures

Outcome measures
Measure
Recombinant Factor XIII
n=6 Participants
One single dose of 35 IU/kg rFXIII was administered as an intravenous (i.v.) injection to each child. The trial included one screening visit, one treatment visit (including pharmacokinetics (PK) assessments up to 24 hours) and 4 follow-up visits (at 7, 14, 21 and 30 days after dosing). Blood samples for PK assessments were drawn pre-dose and up to 30 days after dosing.
Percentage of Subjects With One or More Serious Adverse Events (SAEs)
0 percentage of subjects

SECONDARY outcome

Timeframe: At screening and day 30

Population: Safety analysis population including all 6 children exposed to FXIII. All 6 children were exposed to one dose of trial product.

Outcome measures

Outcome measures
Measure
Recombinant Factor XIII
n=6 Participants
One single dose of 35 IU/kg rFXIII was administered as an intravenous (i.v.) injection to each child. The trial included one screening visit, one treatment visit (including pharmacokinetics (PK) assessments up to 24 hours) and 4 follow-up visits (at 7, 14, 21 and 30 days after dosing). Blood samples for PK assessments were drawn pre-dose and up to 30 days after dosing.
Percentage of Subjects With Development of Anti-rFXIII Antibodies, Including Inhibitors (Neutralising Antibodies Against Factor XIII)
At screening
50 percentage of subjects
Percentage of Subjects With Development of Anti-rFXIII Antibodies, Including Inhibitors (Neutralising Antibodies Against Factor XIII)
Day 30
50 percentage of subjects

SECONDARY outcome

Timeframe: Day 0 and at day 30

Population: Safety analysis population including all 6 children exposed to FXIII. All 6 children were exposed to one dose of trial product.

Outcome measures

Outcome measures
Measure
Recombinant Factor XIII
n=6 Participants
One single dose of 35 IU/kg rFXIII was administered as an intravenous (i.v.) injection to each child. The trial included one screening visit, one treatment visit (including pharmacokinetics (PK) assessments up to 24 hours) and 4 follow-up visits (at 7, 14, 21 and 30 days after dosing). Blood samples for PK assessments were drawn pre-dose and up to 30 days after dosing.
Coagulation Related Parameters - Fibrinogen
Pre-dose
3.26 g/L
Standard Deviation 1.04
Coagulation Related Parameters - Fibrinogen
Post-dose 30 days
3.46 g/L
Standard Deviation 0.82

SECONDARY outcome

Timeframe: Day 0 and day 30

Population: Safety analysis population including all 6 children exposed to FXIII. All 6 children were exposed to one dose of trial product.

Outcome measures

Outcome measures
Measure
Recombinant Factor XIII
n=6 Participants
One single dose of 35 IU/kg rFXIII was administered as an intravenous (i.v.) injection to each child. The trial included one screening visit, one treatment visit (including pharmacokinetics (PK) assessments up to 24 hours) and 4 follow-up visits (at 7, 14, 21 and 30 days after dosing). Blood samples for PK assessments were drawn pre-dose and up to 30 days after dosing.
Coagulation Related Parameters - Activated Partial Thromboplastin Time (aPTT, Seconds)
Pre-dose
33.8 Sec
Standard Deviation 9.0
Coagulation Related Parameters - Activated Partial Thromboplastin Time (aPTT, Seconds)
Post-dose
30.3 Sec
Standard Deviation 5.6

SECONDARY outcome

Timeframe: Day 0 and day 30

Population: Safety analysis population including all 6 children exposed to FXIII. All 6 children were exposed to one dose of trial product.

Outcome measures

Outcome measures
Measure
Recombinant Factor XIII
n=6 Participants
One single dose of 35 IU/kg rFXIII was administered as an intravenous (i.v.) injection to each child. The trial included one screening visit, one treatment visit (including pharmacokinetics (PK) assessments up to 24 hours) and 4 follow-up visits (at 7, 14, 21 and 30 days after dosing). Blood samples for PK assessments were drawn pre-dose and up to 30 days after dosing.
Coagulation Related Parameters - Prothrombin Time (PT) (Seconds)
Pre-dose
12.6 Sec
Standard Deviation 0.7
Coagulation Related Parameters - Prothrombin Time (PT) (Seconds)
Post-dose
12.1 Sec
Standard Deviation 2.3

SECONDARY outcome

Timeframe: Day 0 and day 30

Population: Safety analysis population including all 6 children exposed to FXIII. All 6 children were exposed to one dose of trial product.

Blood samples for clot solubility drawn at each visit (1 hour before and after dose administration). A clot solubility assay was used to screen for FXIII deficiency. The assay is based on the ability of urea to dissolve fibrin clots that have not undergone FXIII-induced stabilization. Normal blood clots generally remain stable for 24 hours or more, while clots in which fibrin molecules have not been cross-linked are soluble within minutes. The outcome of the test is normal (FXIII present; a clot is observed in the test tube) or abnormal (FXIII absent or very low level; no clot in test tube).

Outcome measures

Outcome measures
Measure
Recombinant Factor XIII
n=6 Participants
One single dose of 35 IU/kg rFXIII was administered as an intravenous (i.v.) injection to each child. The trial included one screening visit, one treatment visit (including pharmacokinetics (PK) assessments up to 24 hours) and 4 follow-up visits (at 7, 14, 21 and 30 days after dosing). Blood samples for PK assessments were drawn pre-dose and up to 30 days after dosing.
Clot Solubility Test (Evaluated as Normal/Abnormal)
Normal (pre-dose)
4 participants
Clot Solubility Test (Evaluated as Normal/Abnormal)
Abnormal (pre-dose)
1 participants
Clot Solubility Test (Evaluated as Normal/Abnormal)
Not done (pre-dose)
1 participants
Clot Solubility Test (Evaluated as Normal/Abnormal)
Normal (post-dose)
5 participants
Clot Solubility Test (Evaluated as Normal/Abnormal)
Abnormal (post-dose)
0 participants
Clot Solubility Test (Evaluated as Normal/Abnormal)
Not done (post-dose)
1 participants

SECONDARY outcome

Timeframe: Day 0 and day 30

Population: Safety analysis population including all 6 children exposed to FXIII. All 6 children were exposed to one dose of trial product.

Outcome measures

Outcome measures
Measure
Recombinant Factor XIII
n=6 Participants
One single dose of 35 IU/kg rFXIII was administered as an intravenous (i.v.) injection to each child. The trial included one screening visit, one treatment visit (including pharmacokinetics (PK) assessments up to 24 hours) and 4 follow-up visits (at 7, 14, 21 and 30 days after dosing). Blood samples for PK assessments were drawn pre-dose and up to 30 days after dosing.
Vital Signs - Pulse
pre-dose
95.3 beats/minute
Standard Deviation 14.3
Vital Signs - Pulse
post-dose
113.2 beats/minute
Standard Deviation 10.6

SECONDARY outcome

Timeframe: Day 0 and day 30

Population: Safety analysis population including all 6 children exposed to FXIII. All 6 children were exposed to one dose of trial product.

Outcome measures

Outcome measures
Measure
Recombinant Factor XIII
n=6 Participants
One single dose of 35 IU/kg rFXIII was administered as an intravenous (i.v.) injection to each child. The trial included one screening visit, one treatment visit (including pharmacokinetics (PK) assessments up to 24 hours) and 4 follow-up visits (at 7, 14, 21 and 30 days after dosing). Blood samples for PK assessments were drawn pre-dose and up to 30 days after dosing.
Vital Signs - Blood Pressure (Systolic and Diastolic)
Systolic pre-dose
98.5 mmHg
Standard Deviation 10.9
Vital Signs - Blood Pressure (Systolic and Diastolic)
Systolic post-dose
107.0 mmHg
Standard Deviation 13.7
Vital Signs - Blood Pressure (Systolic and Diastolic)
Diastolic pre-dose
59.8 mmHg
Standard Deviation 12.1
Vital Signs - Blood Pressure (Systolic and Diastolic)
Diastolic post-dose
64.0 mmHg
Standard Deviation 7.6

SECONDARY outcome

Timeframe: From day 0 to day 30

Population: Safety analysis population including all 6 children exposed to FXIII. All 6 children were exposed to one dose of trial product.

Outcome measures

Outcome measures
Measure
Recombinant Factor XIII
n=6 Participants
One single dose of 35 IU/kg rFXIII was administered as an intravenous (i.v.) injection to each child. The trial included one screening visit, one treatment visit (including pharmacokinetics (PK) assessments up to 24 hours) and 4 follow-up visits (at 7, 14, 21 and 30 days after dosing). Blood samples for PK assessments were drawn pre-dose and up to 30 days after dosing.
Physical Examination (Evaluated as Normal/Abnormal)
Normal (post-dose)
5 participants
Physical Examination (Evaluated as Normal/Abnormal)
Abnormal (post-dose)
1 participants
Physical Examination (Evaluated as Normal/Abnormal)
Normal (pre-dose)
6 participants
Physical Examination (Evaluated as Normal/Abnormal)
Abnormal (pre-dose)
0 participants

Adverse Events

Recombinant Factor XIII

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

Serious adverse events

Adverse event data not reported

Other adverse events

Other adverse events
Measure
Recombinant Factor XIII
n=6 participants at risk
One single dose of 35 IU/kg rFXIII was administered as an intravenous (i.v.) injection to each child. The trial included one screening visit, one treatment visit (including pharmacokinetics (PK) assessments up to 24 hours) and 4 follow-up visits (at 7, 14, 21 and 30 days after dosing). Blood samples for PK assessments were drawn pre-dose and up to 30 days after dosing.
General disorders
Pyrexia
16.7%
1/6 • Number of events 1 • Adverse events were collected and reported during the entire trial period; that is from day 0 (the treatment day) to day 30 (last follow-up visit).
Safety analysis population including all 6 children exposed to FXIII. All 6 children were exposed to one dose of trial product.
Musculoskeletal and connective tissue disorders
Pain In Extremity
16.7%
1/6 • Number of events 1 • Adverse events were collected and reported during the entire trial period; that is from day 0 (the treatment day) to day 30 (last follow-up visit).
Safety analysis population including all 6 children exposed to FXIII. All 6 children were exposed to one dose of trial product.

Additional Information

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