Screening for Gaucher Disease and Acid Sphingomyelinase Deficiency

NCT ID: NCT06258577

Last Updated: 2024-04-15

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

NOT_YET_RECRUITING

Total Enrollment

50 participants

Study Classification

OBSERVATIONAL

Study Start Date

2024-05-01

Study Completion Date

2028-12-31

Brief Summary

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High-risk screening for Gaucher disease and Acid Sphingomyelinase Deficiency in patients with splenomegaly and/or thrombocytopenia in Taiwan

Detailed Description

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Late-onset Gaucher disease (GD) present a unique set of challenges compared to their early-onset counterparts. Symptoms may not appear until adulthood, leading to delayed diagnosis and treatment. This delay can result in irreversible damage to affected tissues and organs, such as the liver, spleen, and central nervous system. Additionally, many late-onset GD are underdiagnosed or misdiagnosed due to their rarity and the variability of symptoms. This study is divided into two phases. In the first phase, patients with hepatosplenomegaly of unknown etiology will be initially screened using an electronic medical record database, and in the second phase, laboratory analysis of biomarkers, including Dry blood spot (DBS) for GBA1 enzyme activity, plasma Lyso-GB1 levels and GBA1 gene sequencing, will be performed. Acid sphingomyelinase deficiency (ASMD) is another lysosomal storage disorder that shares symptoms with GD. Consistent with the above screening strategy for GD patients in two phases (DBS for ASM enzyme activity, plasma Lyso-SM levels and ASM gene sequencing). This study will involve 2,000 candidates from electronic healthcare databases, 240 patients from outpatient clinics, and a cohort of 6 GD1/GD3 patients as controls. In conclusion, initial screening for late-onset GD and ASMD can provide patients with treatment opportunities that can improve outcomes for those affected by these rare diseases.

Conditions

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Gaucher Disease

Study Design

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Observational Model Type

CASE_CONTROL

Study Time Perspective

RETROSPECTIVE

Study Groups

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candidates

In the first phase, patients with hepatosplenomegaly of unknown etiology will be initially screened using an electronic medical record database, and in the second phase, laboratory analysis of biomarkers, including Dry blood spot (DBS) for GBA1 enzyme activity, plasma Lyso-GB1 levels and GBA1 gene sequencing, will be performed.

No interventions assigned to this group

control

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No interventions assigned to this group

Eligibility Criteria

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Inclusion Criteria

1. Clinical diagnosis of splenomegaly
2. Clinical diagnosis of thrombocytopenia

Exclusion Criteria

1. Clinical diagnosis of gaucher disease
2. Clinical diagnosis of acid sphingomyelinase
3. Clinical diagnosis of malignant tumors
Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Sanofi

INDUSTRY

Sponsor Role collaborator

Chung-Hsing Wang

OTHER

Sponsor Role lead

Responsible Party

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Chung-Hsing Wang

Attending Physicians

Responsibility Role SPONSOR_INVESTIGATOR

Principal Investigators

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Chung-Hsing Wang

Role: PRINCIPAL_INVESTIGATOR

China Medical University Hospital

Locations

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China Medical University Hospita

Taichung, , Taiwan

Site Status

Countries

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Taiwan

Central Contacts

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Chung-Hsing Wang

Role: CONTACT

0422032798 ext. 14641

Kai-Wen liu

Role: CONTACT

0422032798 ext. 14131

Facility Contacts

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Chung-Hsing Wang

Role: primary

0422032798 ext. 14641

References

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Wan L, Hsu CM, Tsai CH, Lee CC, Hwu WL, Tsai FJ. Mutation analysis of Gaucher disease patients in Taiwan: high prevalence of the RecNciI and L444P mutations. Blood Cells Mol Dis. 2006 May-Jun;36(3):422-5. doi: 10.1016/j.bcmd.2006.02.001. Epub 2006 Mar 20.

Reference Type RESULT
PMID: 16546416 (View on PubMed)

Revel-Vilk S, Fuller M, Zimran A. Value of Glucosylsphingosine (Lyso-Gb1) as a Biomarker in Gaucher Disease: A Systematic Literature Review. Int J Mol Sci. 2020 Sep 28;21(19):7159. doi: 10.3390/ijms21197159.

Reference Type RESULT
PMID: 32998334 (View on PubMed)

Pinto C, Sousa D, Ghilas V, Dardis A, Scarpa M, Macedo MF. Acid Sphingomyelinase Deficiency: A Clinical and Immunological Perspective. Int J Mol Sci. 2021 Nov 28;22(23):12870. doi: 10.3390/ijms222312870.

Reference Type RESULT
PMID: 34884674 (View on PubMed)

McGovern MM, Avetisyan R, Sanson BJ, Lidove O. Disease manifestations and burden of illness in patients with acid sphingomyelinase deficiency (ASMD). Orphanet J Rare Dis. 2017 Feb 23;12(1):41. doi: 10.1186/s13023-017-0572-x.

Reference Type RESULT
PMID: 28228103 (View on PubMed)

Jones SA, McGovern M, Lidove O, Giugliani R, Mistry PK, Dionisi-Vici C, Munoz-Rojas MV, Nalysnyk L, Schecter AD, Wasserstein M. Clinical relevance of endpoints in clinical trials for acid sphingomyelinase deficiency enzyme replacement therapy. Mol Genet Metab. 2020 Sep-Oct;131(1-2):116-123. doi: 10.1016/j.ymgme.2020.06.008. Epub 2020 Jun 24.

Reference Type RESULT
PMID: 32616389 (View on PubMed)

Loftus WK, Metreweli C. Normal splenic size in a Chinese population. J Ultrasound Med. 1997 May;16(5):345-7.

Reference Type RESULT
PMID: 9315173 (View on PubMed)

Other Identifiers

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CMUH112-REC2-098

Identifier Type: -

Identifier Source: org_study_id

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