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CompletedNCT04470713RETRIEVEUpdated Nov 8, 2021

Natural History Study for Pediatric Patients With Early Onset of Either GM1 Gangliosidosis, GM2 Gangliosidoses, or Gaucher Disease Type 2

An observational study in GM1 Gangliosidosis, GM2 Gangliosidosis and Gaucher Disease, Type 2, sponsored by Idorsia Pharmaceuticals Ltd.. Completed at 17 sites in 10 countries. Open to participants aged 0 Months and older. Per ClinicalTrials.gov, last updated 2021-11-08.

Sponsored by Idorsia Pharmaceuticals Ltd. · Observational

Study type
Observational
Model
Case-only
Time perspective
Other
Enrollment
226
Ages
0 Months and older
Sex
All
01

Study summary

This study is being conducted to better understand the natural course of GM1 gangliosidosis, GM2 gangliosidoses and Gaucher disease Type 2 (GD2). Information is planned to be gathered on at least 180 patients with GM1 gangliosidosis, GM2 gangliosidoses, and Gaucher Disease type 2. Retrospective data collection is planned for at least 150 deceased patients (Group A). Group B is for patients alive at the time of enrollment. In Group B it is planned to prospectively collect more comprehensive data from at least 30 patients. The purpose of this study is to collect relevant information for a adequate design of a potential subsequent research program in these diseases.

In this study no therapy is being offered.

02

Conditions studied

  • GM1 Gangliosidosis
  • GM2 Gangliosidosis
  • Gaucher Disease, Type 2
  • Tay-Sachs Disease
  • AB Variant Gangliosidosis GM2
  • Sandhoff Disease
03

Who can participate

Ages eligible
0 Months and older
Sexes eligible
All
Accepts healthy volunteers
No
Sampling method
Non-probability sample

Study population

This study will be conducted in hospitals/clinical centers managing pediatric patients with GM1 gangliosidosis, GM2 gangliosidoses, and/or Gaucher Disease type 2 across several countries in North America, South America, and Europe. In order to minimize the patient/data selection bias, the centers are asked to include all eligible patients from their center.

Eligibility criteria

Inclusion Criteria:

  • Patient with either GM1 gangliosidosis, GM2 gangliosidoses (Tay-Sachs, Sandhoff, or AB Variant), or Gaucher Disease Type 2.
  • Diagnosis confirmed by either biochemical (enzyme activity) or genetic testing, or both.
  • Date of birth on or after 1 January 2000.
  • Onset of first neurological symptom within 24 months of age.
  • Informed consent of parent or legal guardian as required by local law.
04

Study design

Observational model
Case-only
Time perspective
Other
Enrollment
226 participants (actual)
Patient registry
No

Groups and cohorts

  • Group A - Retrospective data collection

    Participants with a confirmed diagnosis, either deceased patients or patients whose survival status is not known at enrollment.

  • Group B - Prospective data collection

    Participants who are alive at enrollment. Data collection is retrospective for the time between birth and enrollment visit, and data collection is prospective from the enrollment visit onwards. Visits are performed as per local standard of care.

05

What researchers measure

Primary outcomes

  1. Survival of pediatric patients with early onset of GM1 gangliosidosis, GM2 gangliosidoses, and Gaucher Disease type 2

    Time frame: 2.5 years

Other outcomes

  1. Epidemiological data available from medical records

    Patients' medical record data such as date of diagnosis, the date of appearance of first neurological symptom, dates of gain or loss of specific abilities (e.g. ability to sit) will be collected, if available.

    Time frame: 2.5 years

06

Study locations

17 sites
  • Ann & Robert H. Lurie Children's Hospital of Chicago
    Chicago, Illinois 60611, United States
  • Mayo Clinic - Rochester
    Rochester, Minnesota 55905, United States
  • Lysosomal and Rare Disorders Research and Treatment Center
    Fairfax, Virginia 22030, United States
  • UCL Cliniques Universitaires Saint-Luc
    Bruxelles, 1020, Belgium
  • Hospital de Clínicas de Porto Alegre - HCPA
    Porto Alegre, 90035-903, Brazil
  • AP-HP - Hôpitaux Universitaires Est Parisien
    Paris, 75012, France
  • SphinCS GmbH
    Hochheim, 65239, Germany
  • Azienda Ospedaliero Universitaria Meyer
    Florence, 50139, Italy
  • Fondazione I.R.C.C.S. Istituto Neurologico Carlo Besta
    Milano, 20133, Italy
  • Centro Hospitalar Universitario Lisboa Norte, EPE
    Lisboa, 1649-035, Portugal
  • Centro Universitario Hospitalar de São João, EPE
    Porto, 4200-319, Portugal
  • Hospital Sant Joan de Deu
    Barcelona, 08950, Spain
  • Quirónsalud
    Zaragoza, 50006, Spain
  • Universitätsspital Bern Inselspital
    Bern, CH-3010, Switzerland
  • Universitäts-Kinderspital Zürich
    Zürich, CH-8032, Switzerland
  • University Hospitals Birmingham NHS Foundation Trust
    Birmingham, B4 6NH, United Kingdom
  • Great Ormond Street Hospital for Children NHS Found. Trust
    London, WC1N 3JH, United Kingdom
07

References and documents

Individual participant data

Plan to share: No

No publications or documents are linked to this record.

08

Registry details

Key details

Study ID
NCT04470713
Lead sponsor
Idorsia Pharmaceuticals Ltd.
Responsible party
Sponsor
First posted
Jul 14, 2020
Start date
Jul 31, 2019
Primary completion
Oct 30, 2021
Completion
Oct 30, 2021
Last update
Nov 8, 2021

Study contacts

Clinical Trials
study director · Idorsia Pharmaceuticals Ltd.

Oversight

Data monitoring committee
No
FDA-regulated drug
No
FDA-regulated device
No
View the source record on ClinicalTrials.gov ↗

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This study is completed, as verified in Nov 2021. You cannot join it, but the record below documents what was studied.

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