Trial Outcomes & Findings for Study of Velaglucerase Alfa Enzyme Replacement Therapy in Japanese Patients With Gaucher Disease (NCT NCT01614574)

NCT ID: NCT01614574

Last Updated: 2021-06-28

Results Overview

Recruitment status

COMPLETED

Study phase

PHASE3

Target enrollment

6 participants

Primary outcome timeframe

Baseline to week 51

Results posted on

2021-06-28

Participant Flow

Participant milestones

Participant milestones
Measure
VPRIV® (15-60 U/kg)
Administered as an intravenous (IV) infusion over a 60 minute period.
Overall Study
STARTED
6
Overall Study
COMPLETED
6
Overall Study
NOT COMPLETED
0

Reasons for withdrawal

Withdrawal data not reported

Baseline Characteristics

Study of Velaglucerase Alfa Enzyme Replacement Therapy in Japanese Patients With Gaucher Disease

Baseline characteristics by cohort

Baseline characteristics by cohort
Measure
VPRIV® (15-60 U/kg)
n=6 Participants
Administered as an intravenous (IV) infusion over a 60 minute period.
Age, Continuous
20 Years
STANDARD_DEVIATION 10.62 • n=5 Participants
Age, Customized
<=18 years
4 Participants
n=5 Participants
Age, Customized
Between 18 and 65 years
2 Participants
n=5 Participants
Sex: Female, Male
Female
1 Participants
n=5 Participants
Sex: Female, Male
Male
5 Participants
n=5 Participants
Region of Enrollment
JAPAN
6 Participants
n=5 Participants
Hemoglobin Concentration
13.77 (g/dL)
STANDARD_DEVIATION 0.954 • n=5 Participants
Platelet Count
182.9 (x 10^9 platelets/L)
STANDARD_DEVIATION 32.05 • n=5 Participants
Normalized liver volume
1.92 (% of Body Weight)
STANDARD_DEVIATION 0.342 • n=5 Participants
Normalized spleen volume
0.39 (% of Body Weight)
STANDARD_DEVIATION 0.067 • n=5 Participants
Plasma chitotriosidase
1243.7 (nmol/mL/h)
STANDARD_DEVIATION 344.46 • n=5 Participants
CCL18 levels
134.8 (ng/mL)
STANDARD_DEVIATION 132.26 • n=5 Participants

PRIMARY outcome

Timeframe: Baseline to week 51

Outcome measures

Outcome measures
Measure
VPRIV® (15-60 U/kg)
n=6 Participants
Administered as an intravenous (IV) infusion over a 60 minute period.
Number of Severe Adverse Events (SAE)
1 events

PRIMARY outcome

Timeframe: Baseline to week 51

Outcome measures

Outcome measures
Measure
VPRIV® (15-60 U/kg)
n=6 Participants
Administered as an intravenous (IV) infusion over a 60 minute period.
Number of Treatment Emergent Adverse Events (TEAE)
31 events

PRIMARY outcome

Timeframe: Baseline to week51

Outcome measures

Outcome measures
Measure
VPRIV® (15-60 U/kg)
n=6 Participants
Administered as an intravenous (IV) infusion over a 60 minute period.
Development of Anti-velaglucerase Alfa Antibody
Positive (Baseline)
0 participants
Development of Anti-velaglucerase Alfa Antibody
Negative (Baseline)
6 participants
Development of Anti-velaglucerase Alfa Antibody
Positive (Week 51)
0 participants
Development of Anti-velaglucerase Alfa Antibody
Negative (Week 51)
6 participants

PRIMARY outcome

Timeframe: Baseline to week 51

Outcome measures

Outcome measures
Measure
VPRIV® (15-60 U/kg)
n=6 Participants
Administered as an intravenous (IV) infusion over a 60 minute period.
Number of Infusion- Related Adverse Events
2 events

PRIMARY outcome

Timeframe: Baseline to week 51

Outcome measures

Outcome measures
Measure
VPRIV® (15-60 U/kg)
n=6 Participants
Administered as an intravenous (IV) infusion over a 60 minute period.
Number of Patients With Concomitant Medication
6 participants

SECONDARY outcome

Timeframe: Baseline to week 51

Outcome measures

Outcome measures
Measure
VPRIV® (15-60 U/kg)
n=6 Participants
Administered as an intravenous (IV) infusion over a 60 minute period.
Change From Baseline in Hemoglobin Concentration
0.05 (g/dL)
Standard Deviation 0.729

SECONDARY outcome

Timeframe: Baseline to week 51

Outcome measures

Outcome measures
Measure
VPRIV® (15-60 U/kg)
n=6 Participants
Administered as an intravenous (IV) infusion over a 60 minute period.
Change From Baseline in Platelet Count
13.8 (x 10^9 platelets/L)
Standard Deviation 35.75

SECONDARY outcome

Timeframe: Baseline to week 51

Outcome measures

Outcome measures
Measure
VPRIV® (15-60 U/kg)
n=6 Participants
Administered as an intravenous (IV) infusion over a 60 minute period.
Change From Baseline in Liver Volume, Normalized to Body Weight
0.05 (% of Body Weight)
Standard Deviation 0.148

SECONDARY outcome

Timeframe: Baseline to week 51

Outcome measures

Outcome measures
Measure
VPRIV® (15-60 U/kg)
n=6 Participants
Administered as an intravenous (IV) infusion over a 60 minute period.
Change From Baseline in Spleen Volume, Normalized to Body Weight
0.39 (% of Body Weight)
Standard Deviation 0.067

SECONDARY outcome

Timeframe: Baseline to week 51

Outcome measures

Outcome measures
Measure
VPRIV® (15-60 U/kg)
n=6 Participants
Administered as an intravenous (IV) infusion over a 60 minute period.
Change From Baseline in Plasma Chitotriosidase Levels
-47.3 (nmol/mL/h)
Standard Deviation 64.44

SECONDARY outcome

Timeframe: Baseline to week 51

Outcome measures

Outcome measures
Measure
VPRIV® (15-60 U/kg)
n=6 Participants
Administered as an intravenous (IV) infusion over a 60 minute period.
Change From Baseline in CCL18 Levels
5.2 (ng/mL)
Standard Deviation 10.59

Adverse Events

VPRIV® (15-60 U/kg)

Serious events: 1 serious events
Other events: 6 other events
Deaths: 0 deaths

Serious adverse events

Serious adverse events
Measure
VPRIV® (15-60 U/kg)
n=6 participants at risk
Administered as an intravenous (IV) infusion over a 60 minute period.
Eye disorders
RETINAL DETACHMENT
16.7%
1/6 • Number of events 1

Other adverse events

Other adverse events
Measure
VPRIV® (15-60 U/kg)
n=6 participants at risk
Administered as an intravenous (IV) infusion over a 60 minute period.
Eye disorders
RETINOPATHY PROLIFERATIVE
16.7%
1/6 • Number of events 1
Eye disorders
VISUAL ACUITY REDUCED
16.7%
1/6 • Number of events 1
Gastrointestinal disorders
ABDOMINAL PAIN UPPER
16.7%
1/6 • Number of events 1
Gastrointestinal disorders
CHEILITIS
16.7%
1/6 • Number of events 1
Gastrointestinal disorders
ENTERITIS
16.7%
1/6 • Number of events 1
Gastrointestinal disorders
NAUSEA
16.7%
1/6 • Number of events 1
Gastrointestinal disorders
VOMITING
16.7%
1/6 • Number of events 1
General disorders
OEDEMA PERIPHERAL
16.7%
1/6 • Number of events 1
General disorders
SUBMANDIBULAR MASS
16.7%
1/6 • Number of events 1
Infections and infestations
GASTROENTERITIS
16.7%
1/6 • Number of events 2
Infections and infestations
HAND-FOOT-AND-MOUTH DISEASE
16.7%
1/6 • Number of events 1
Infections and infestations
HORDEOLUM
16.7%
1/6 • Number of events 1
Infections and infestations
NASOPHARYNGITIS
100.0%
6/6 • Number of events 9
Infections and infestations
OTITIS MEDIA
16.7%
1/6 • Number of events 1
Injury, poisoning and procedural complications
CONTUSION
50.0%
3/6 • Number of events 3
Injury, poisoning and procedural complications
JOINT SPRAIN
16.7%
1/6 • Number of events 1
Injury, poisoning and procedural complications
LIMB INJURY
16.7%
1/6 • Number of events 1
Musculoskeletal and connective tissue disorders
ARTHRALGIA
33.3%
2/6 • Number of events 2
Nervous system disorders
HEADACHE
16.7%
1/6 • Number of events 1
Nervous system disorders
LOSS OF CONSCIOUSNESS
16.7%
1/6 • Number of events 1
Nervous system disorders
MYOCLONUS
16.7%
1/6 • Number of events 1
Psychiatric disorders
ADJUSTMENT DISORDER
16.7%
1/6 • Number of events 1
Reproductive system and breast disorders
MENSTRUATION IRREGULAR
16.7%
1/6 • Number of events 1
Respiratory, thoracic and mediastinal disorders
COUGH
16.7%
1/6 • Number of events 1
Respiratory, thoracic and mediastinal disorders
EPISTAXIS
16.7%
1/6 • Number of events 2
Respiratory, thoracic and mediastinal disorders
PHARYNGOLARYNGEAL PAIN
16.7%
1/6 • Number of events 1
Respiratory, thoracic and mediastinal disorders
TACHYPNOEA
16.7%
1/6 • Number of events 1
Skin and subcutaneous tissue disorders
ACNE
16.7%
1/6 • Number of events 1
Skin and subcutaneous tissue disorders
ALOPECIA
16.7%
1/6 • Number of events 1
Skin and subcutaneous tissue disorders
ECZEMA
16.7%
1/6 • Number of events 1
Skin and subcutaneous tissue disorders
URTICARIA
16.7%
1/6 • Number of events 1

Additional Information

Study Director

Shire

Phone: +1 866 842 5335

Results disclosure agreements

  • Principal investigator is a sponsor employee Shire's agreements with investigators vary. All agreements provide Shire the right to embargo communications regarding trial results prior to public release for a period ≤180 days from the time submitted to Shire for review. Shire does not prohibit publication, but can require the removal of confidential information (excluding trial results) and can request postponement of a single-center publication until after disclosure of the trial's multi-center publication
  • Publication restrictions are in place

Restriction type: OTHER