Longitudinal Studies of the Glycoproteinoses

NCT ID: NCT01891422

Last Updated: 2023-09-13

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

COMPLETED

Total Enrollment

100 participants

Study Classification

OBSERVATIONAL

Study Start Date

2009-08-31

Study Completion Date

2020-07-31

Brief Summary

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The glycoproteinoses are among the most rare and least researched of the lysosomal diseases. The diseases include aspartylglucosaminuria, fucosidosis, galactosialidosis, alpha-mannosidosis, beta-mannosidosis, mucolipidosis II, mucolipidosis III, Schindler disease, and sialidosis. Longitudinal studies of these conditions are needed in order to better define how common the diseases are, identify clinical features which could contribute to early diagnoses, detail progression of the diseases, assess the supportive therapies currently used, and identify potential treatments. Laboratory tests will evaluate metabolic and genetic defects found in participants' blood and urine samples.

Detailed Description

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This is a longitudinal study of 100 individuals diagnosed with any one of the nine glycoproteinoses. Because of the small number of individuals diagnosed with these diseases, participants will be strongly encouraged to be evaluated in person at a study site, but inability to travel to a study site will not exclude a person from participating. This non-interventional study will also collect medical information about participants through questionnaire, phone interviews, and review of medical records regarding the person's usual medical care, including lab testing and x-rays or other imaging studies.

Participants who are evaluated at the study center will have a physical examination performed by a clinical geneticist and neuropsychological studies administered by the study psychologist. Neuropsychological studies assess intelligence, learning abilities, language skills, and ability to participate in day to day activities of life. Participants seen at the study center will have skeletal x-rays performed to evaluate the impact of the disease on the participant's bones.

Every participant will complete (or have a care provider complete for them)

* A questionnaire about their birth, development, and medical history
* An interview with study personnel (in person or via telephone)
* Follow up interviews on at least an annual basis to update the medical history

Each participant will be asked to

* Give a blood sample
* Give a urine sample
* Some participants may be asked to give a skin biopsy.

Conditions

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Aspartylglucosaminuria Fucosidosis Galactosialidosis Alpha Mannosidosis Beta Mannosidosis Mucolipidosis II Mucolipidosis III Schindler Disease Sialidosis

Study Design

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Observational Model Type

COHORT

Study Time Perspective

OTHER

Interventions

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Non-interventional.

Intervention Type OTHER

Eligibility Criteria

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Inclusion Criteria

Be an individual of any age diagnosed with one of the following conditions

* Aspartylglucosaminuria
* Fucosidosis
* Galactosialidosis
* alpha mannosidosis
* beta mannosidosis
* Mucolipidosis II
* Mucolipidosis III
* Schindler disease
* Sialidosis

Exclusion Criteria

* not diagnosed with one of the nine glycoproteinoses listed above.
Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Greenwood Genetic Center

OTHER

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Sara Cathey, MD

Role: PRINCIPAL_INVESTIGATOR

Greenwood Genetic Center

Locations

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Greenwood Genetic Center

North Charleston, South Carolina, United States

Site Status

Countries

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United States

References

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Kerr DA, Memoli VA, Cathey SS, Harris BT. Mucolipidosis type III alpha/beta: the first characterization of this rare disease by autopsy. Arch Pathol Lab Med. 2011 Apr;135(4):503-10. doi: 10.5858/2010-0236-CR.1.

Reference Type BACKGROUND
PMID: 21466370 (View on PubMed)

David-Vizcarra G, Briody J, Ault J, Fietz M, Fletcher J, Savarirayan R, Wilson M, McGill J, Edwards M, Munns C, Alcausin M, Cathey S, Sillence D. The natural history and osteodystrophy of mucolipidosis types II and III. J Paediatr Child Health. 2010 Jun;46(6):316-22. doi: 10.1111/j.1440-1754.2010.01715.x. Epub 2010 Mar 29.

Reference Type BACKGROUND
PMID: 20367762 (View on PubMed)

Cathey SS, Leroy JG, Wood T, Eaves K, Simensen RJ, Kudo M, Stevenson RE, Friez MJ. Phenotype and genotype in mucolipidoses II and III alpha/beta: a study of 61 probands. J Med Genet. 2010 Jan;47(1):38-48. doi: 10.1136/jmg.2009.067736. Epub 2009 Jul 16.

Reference Type RESULT
PMID: 19617216 (View on PubMed)

Related Links

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http://www.ggc.org

Greenwood Genetic Center

Other Identifiers

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GGC75

Identifier Type: -

Identifier Source: org_study_id

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