Trial Outcomes & Findings for Auto-antibodies Prevalence and CD1 Role in Gaucher Disease (NCT NCT02650219)

NCT ID: NCT02650219

Last Updated: 2016-03-23

Results Overview

acidβ-glucosidase enzyme testing : a lower than 15% of mean normal activity is considered to be diagnostic. Decreased enzyme levels will often be confirmed by genetic testing. Numerous different mutations occur; GB1 mutation analyses is sometimes necessary to confirm the diagnosis.

Recruitment status

COMPLETED

Target enrollment

60 participants

Primary outcome timeframe

baseline

Results posted on

2016-03-23

Participant Flow

from January 2010 to April 2011, 40 GD1 patients and 20 healthy volunteers (control group) were included in the study in the 12 inclusions centers (medical clinic or hopsital)

Participant milestones

Participant milestones
Measure
Gaucher Disease Type 1
Inclusion criteria: * Adult patients \>= 18 years old * Gaucher disease type 1, proved by low betaglucosidase, with or without treatment * Patients must have read, understood and signed informed consent. intervention : genetic analyses genetic analyses
Control
healthy subjects intervention: genetic analyses genetic analyses
Overall Study
STARTED
40
20
Overall Study
COMPLETED
40
20
Overall Study
NOT COMPLETED
0
0

Reasons for withdrawal

Withdrawal data not reported

Baseline Characteristics

Auto-antibodies Prevalence and CD1 Role in Gaucher Disease

Baseline characteristics by cohort

Baseline characteristics by cohort
Measure
Gaucher Disease Type 1
n=40 Participants
Inclusion criteria: * Adult patients \>= 18 years old * Gaucher disease type 1, proved by low betaglucosidase, with or without treatment * Patients must have read, understood and signed informed consent. intervention : genetic analyses genetic analyses
Control
n=20 Participants
healthy subjects intervention: genetic analyses genetic analyses
Total
n=60 Participants
Total of all reporting groups
Age, Categorical
<=18 years
0 Participants
n=5 Participants
0 Participants
n=7 Participants
0 Participants
n=5 Participants
Age, Categorical
Between 18 and 65 years
31 Participants
n=5 Participants
17 Participants
n=7 Participants
48 Participants
n=5 Participants
Age, Categorical
>=65 years
9 Participants
n=5 Participants
3 Participants
n=7 Participants
12 Participants
n=5 Participants
Age, Continuous
52.4 YEARS
STANDARD_DEVIATION 13.4 • n=5 Participants
47.3 YEARS
STANDARD_DEVIATION 15.4 • n=7 Participants
50.7 YEARS
STANDARD_DEVIATION 14.2 • n=5 Participants
Sex: Female, Male
Female
22 Participants
n=5 Participants
13 Participants
n=7 Participants
35 Participants
n=5 Participants
Sex: Female, Male
Male
18 Participants
n=5 Participants
7 Participants
n=7 Participants
25 Participants
n=5 Participants
Region of Enrollment
France
40 participants
n=5 Participants
20 participants
n=7 Participants
60 participants
n=5 Participants

PRIMARY outcome

Timeframe: baseline

acidβ-glucosidase enzyme testing : a lower than 15% of mean normal activity is considered to be diagnostic. Decreased enzyme levels will often be confirmed by genetic testing. Numerous different mutations occur; GB1 mutation analyses is sometimes necessary to confirm the diagnosis.

Outcome measures

Outcome measures
Measure
Gaucher Disease Type 1
n=40 Participants
Inclusion criteria: * Adult patients \>= 18 years old * Gaucher disease type 1, proved by low betaglucosidase, with or without treatment * Patients must have read, understood and signed informed consent. intervention : genetic analyses genetic analyses
Control
n=20 Participants
healthy subjects intervention: genetic analyses genetic analyses
Number of Patients With GD Diagnosis Confirmed by : Enzyme Testing of acidβ-glucosidase Activity Activity <15% in Blood Leucocytes Completed When Necsssary by GB1 Mutation Analyses (Analyses From Samples)
40 participant
0 participant

SECONDARY outcome

Timeframe: Baseline

Population: splenectomy testing

data available from medical record of the patients

Outcome measures

Outcome measures
Measure
Gaucher Disease Type 1
n=40 Participants
Inclusion criteria: * Adult patients \>= 18 years old * Gaucher disease type 1, proved by low betaglucosidase, with or without treatment * Patients must have read, understood and signed informed consent. intervention : genetic analyses genetic analyses
Control
n=20 Participants
healthy subjects intervention: genetic analyses genetic analyses
Number of Patients With : Splenectomy and/or Bone Events and/or Pulmonary Hypertension and/or Specific Treatment and Non-specific (Medical History,Physiological Parameters and Questionnaire)
15 participants
0 participants

SECONDARY outcome

Timeframe: Baseline

Features usually associated with auto immune disease- data available from medical record of the patients

Outcome measures

Outcome data not reported

SECONDARY outcome

Timeframe: baseline

Population: antinuclear autoantibodies

data available from biological analyses (blood samples)

Outcome measures

Outcome measures
Measure
Gaucher Disease Type 1
n=40 Participants
Inclusion criteria: * Adult patients \>= 18 years old * Gaucher disease type 1, proved by low betaglucosidase, with or without treatment * Patients must have read, understood and signed informed consent. intervention : genetic analyses genetic analyses
Control
n=20 Participants
healthy subjects intervention: genetic analyses genetic analyses
Number of Patients With : Antinuclear and/or Anti-SSa and/or Anti-SSb and/or Anti-RNP and/or Anti-DNA and/or Anti-Sm and/or Anticardiolipid and/or Anti β2Gp1 and/or Antiganglioside Autoantibodies (Genetics Analyses From Blood Samples)
10 participants
3 participants

Adverse Events

Gaucher Disease Type 1

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

Control

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

Serious adverse events

Adverse event data not reported

Other adverse events

Adverse event data not reported

Additional Information

Dr Christine Serratrice

St Joseph Hospital Marseille

Phone: 0041798731474

Results disclosure agreements

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