Intra-Erythrocyte Dexamethasone Sodium Phosphate in Ataxia Teleangiectasia Patients (IEDAT01)
Primary Purpose
Nervous System Disorder, Genetic Syndrome
Status
Completed
Phase
Phase 2
Locations
Italy
Study Type
Interventional
Intervention
Dexamethasone
Sponsored by
About this trial
This is an interventional treatment trial for Nervous System Disorder focused on measuring Ataxia Teleangiectasia, AT, Ery-Dex, Dexamethasone, Dexamethasone sodium phosphate
Eligibility Criteria
Inclusion Criteria:
- neurological signs of AT
- patients in autonomous gait or helped by a support
- proven molecular diagnosis of AT
- Males and females aged > 3 years
- Body weight >15 kg
- Plasma levels of Lymphocytes CD4+/mm3 > 500 (for patients aged 3-6 years) or > 200 (older than 6 years)
Exclusion Criteria:
- Current or previous neoplastic disease
- History of severe impairment of the immunological system
- Chronic conditions representing a contraindication to the use of steroid drugs
- Non compliance with the study request
- Any previous steroid assumption within 30 days before starting Ery-Dex
Sites / Locations
- Spedali Civili
- University La Sapienza
Arms of the Study
Arm 1
Arm Type
Experimental
Arm Label
Ery-Dex
Arm Description
Patients treated with monthly treatment of Ery-Dex (dexamethasone sodium phosphate encapsulated in autologous erythrocytes)
Outcomes
Primary Outcome Measures
Changes in Neurological symptoms assessed by using ICARS score
ICARS is a 100-point semiquantitative scale offering a compartimentalised quantification of 4 subscores: Posture and Gait disorders; Kinetic functions; Speech Disorders and Oculomotor Disorders, for a possible total of 100 (highest score corresponds to the worst status of the patient).
Secondary Outcome Measures
Assessment of safety
Evaluate the effect of Ery-Dex on treatment emergent adverse events including Serious Adverse Events (SAEs) and laboratory parameters
Vineland adaptive behaviour scale (VABS)
To evaluate the effect of Ery-Dexon adaptive behavior measured by VABS
Full Information
1. Study Identification
Unique Protocol Identification Number
NCT01255358
Brief Title
Intra-Erythrocyte Dexamethasone Sodium Phosphate in Ataxia Teleangiectasia Patients
Acronym
IEDAT01
Official Title
Evaluations of Effects of Intra-Erythrocyte Dexamethasone Sodium Phosphate on Neurological Symptoms in Ataxia Teleangiectasia Patients
Study Type
Interventional
2. Study Status
Record Verification Date
December 2011
Overall Recruitment Status
Completed
Study Start Date
February 2011 (undefined)
Primary Completion Date
September 2011 (Actual)
Study Completion Date
December 2011 (Actual)
3. Sponsor/Collaborators
Responsible Party, by Official Title
Sponsor
Name of the Sponsor
Erydel
4. Oversight
Data Monitoring Committee
No
5. Study Description
Brief Summary
Multi-centre, single arm, open label, 6 months, phase II study to evaluate the effect of Ery-Dex in improving Central Nervous System (CNS) symptoms in patients with Ataxia Teleangiectasia (AT). The study consists of a screening period (max duration of 30 days) and a treatment period (duration 6 months).
Detailed Description
The study has the aim to evaluate the improvement in CNS symptoms measured by International Co-operative Ataxia Rating Scale (ICARS) in patients with AT, during a period of treatment with Ery-Dex (dexamethasone sodium phosphate ex vivo encapsulated into human autologous erythrocytes).
6. Conditions and Keywords
Primary Disease or Condition Being Studied in the Trial, or the Focus of the Study
Nervous System Disorder, Genetic Syndrome
Keywords
Ataxia Teleangiectasia, AT, Ery-Dex, Dexamethasone, Dexamethasone sodium phosphate
7. Study Design
Primary Purpose
Treatment
Study Phase
Phase 2
Interventional Study Model
Single Group Assignment
Masking
None (Open Label)
Allocation
N/A
Enrollment
22 (Actual)
8. Arms, Groups, and Interventions
Arm Title
Ery-Dex
Arm Type
Experimental
Arm Description
Patients treated with monthly treatment of Ery-Dex (dexamethasone sodium phosphate encapsulated in autologous erythrocytes)
Intervention Type
Drug
Intervention Name(s)
Dexamethasone
Other Intervention Name(s)
Dexamethasone sodium phosphate, Dex 21P
Intervention Description
dexamethasone encapsulated in human erythrocytes. One infusion per month of about 10-15 mg of dexamethasone. The treatment covers 1 month of therapy-
Primary Outcome Measure Information:
Title
Changes in Neurological symptoms assessed by using ICARS score
Description
ICARS is a 100-point semiquantitative scale offering a compartimentalised quantification of 4 subscores: Posture and Gait disorders; Kinetic functions; Speech Disorders and Oculomotor Disorders, for a possible total of 100 (highest score corresponds to the worst status of the patient).
Time Frame
At the end of 6 months of treatment
Secondary Outcome Measure Information:
Title
Assessment of safety
Description
Evaluate the effect of Ery-Dex on treatment emergent adverse events including Serious Adverse Events (SAEs) and laboratory parameters
Time Frame
During the whole treatment period
Title
Vineland adaptive behaviour scale (VABS)
Description
To evaluate the effect of Ery-Dexon adaptive behavior measured by VABS
Time Frame
At the end of the study
10. Eligibility
Sex
All
Minimum Age & Unit of Time
3 Years
Accepts Healthy Volunteers
No
Eligibility Criteria
Inclusion Criteria:
neurological signs of AT
patients in autonomous gait or helped by a support
proven molecular diagnosis of AT
Males and females aged > 3 years
Body weight >15 kg
Plasma levels of Lymphocytes CD4+/mm3 > 500 (for patients aged 3-6 years) or > 200 (older than 6 years)
Exclusion Criteria:
Current or previous neoplastic disease
History of severe impairment of the immunological system
Chronic conditions representing a contraindication to the use of steroid drugs
Non compliance with the study request
Any previous steroid assumption within 30 days before starting Ery-Dex
Overall Study Officials:
First Name & Middle Initial & Last Name & Degree
Luciana Chessa, MD
Organizational Affiliation
A.O. Sant'Andrea Rome Italy
Official's Role
Principal Investigator
Facility Information:
Facility Name
Spedali Civili
City
Brescia
ZIP/Postal Code
25123
Country
Italy
Facility Name
University La Sapienza
City
Rome
ZIP/Postal Code
00185
Country
Italy
12. IPD Sharing Statement
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Intra-Erythrocyte Dexamethasone Sodium Phosphate in Ataxia Teleangiectasia Patients
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