Immune Biomarkers Related to Bone Pathology in Patients With Type 1 Gaucher Disease

NCT ID: NCT04055831

Last Updated: 2019-08-15

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

UNKNOWN

Total Enrollment

40 participants

Study Classification

OBSERVATIONAL

Study Start Date

2019-05-15

Study Completion Date

2020-05-15

Brief Summary

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Bone-related problems represent the principal unmet medical need in Gaucher disease (GD). 75% of GD type 1 patients develop skeletal complications, including bone remodeling defects, osteopenia, osteoporosis, marrow infiltration, avascular necrosis, and osteolysis. However, the underlying cellular/molecular basis of bone involvement and related complications in GD are not fully known. Neither are there any bone-specific markers associated with individual bone pathology. Early diagnosis of bone disease is the key issue for planning individual therapy to prevent and reverse bone disease in GD.

Detailed Description

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This clinical observational study is designed to identify specific biomarkers for bone involvement in patients with GD1 with decreased bone density and/or bone structural abnormalities

Aims:

1. Identify novel immune cell surface and biochemical markers in peripheral blood correlating with bone involvement in GD.
2. Assess the correlation between cytokine levels in peripheral blood and the severity of bone involvement in GD.
3. Assess the relationship between glycosphingolipids accumulation and macrophage activation with specific bone markers and GD severity.

Conditions

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Gaucher Disease Type 1

Study Design

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Observational Model Type

CASE_CONTROL

Study Time Perspective

PROSPECTIVE

Study Groups

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GD1 subjects with no bone complications

1\. GD1 subjects with no bone complications (n=10)

No interventions assigned to this group

GD1 patients with mild bone complication

2\. GD1 subjects with mild bone complications

No interventions assigned to this group

GD1 with severe bone complications

3\. GD1 subjects with severe bone complications

No interventions assigned to this group

No bone disease

Controls with no known bone disease (n=10)

No interventions assigned to this group

Eligibility Criteria

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Inclusion Criteria

* To be enrolled in this study the subject must meet the following criteria

1. Subject is greater than 16 years old but not older than 90 years
2. Signed Informed Consent/Assent
3. Subject is able and willing to sign informed consent or assent
4. If the subject has GD1, the must have a confirmed diagnosis of Gaucher disease by

* GCase enzyme activity
* DNA analysis demonstrating pathogenic variants in the GBA gene

Exclusion Criteria

* a) Have evidence of hepatitis B, hepatitis C infection or any other chronic infectious disease b) Be pregnant or breastfeeding
Minimum Eligible Age

16 Years

Maximum Eligible Age

90 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

Yes

Sponsors

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Lysosomal and Rare Disorders Research and Treatment Center, Inc.

OTHER

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Ozlem Goker-Alpan, MD

Role: PRINCIPAL_INVESTIGATOR

Lysosomal and Rare Disorders Research and Treatment Center

Locations

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LDRTC

Fairfax, Virginia, United States

Site Status RECRUITING

Countries

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United States

Central Contacts

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Jacqueline Fikry

Role: CONTACT

571-732-4575

Margarita Ivanova, PhD

Role: CONTACT

5715296724

Facility Contacts

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Ozlem Goker-Alpan, MD

Role: primary

703-261-6220

Margarita M Ivanova, PhD

Role: backup

7032616220 ext. 5022957709

References

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Ivanova MM, Dao J, Kasaci N, Friedman A, Noll L, Goker-Alpan O. Wnt signaling pathway inhibitors, sclerostin and DKK-1, correlate with pain and bone pathology in patients with Gaucher disease. Front Endocrinol (Lausanne). 2022 Nov 24;13:1029130. doi: 10.3389/fendo.2022.1029130. eCollection 2022.

Reference Type DERIVED
PMID: 36506070 (View on PubMed)

Other Identifiers

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19-LDRTC-01

Identifier Type: -

Identifier Source: org_study_id

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