ID

45155

Beschrijving

Principal Investigator: Maureen D. Mayes, MD, MPH, University of Texas Health Science Center, Houston, TX, USA MeSH: Systemic Sclerosis,Scleroderma, Systemic https://www.ncbi.nlm.nih.gov/projects/gap/cgi-bin/study.cgi?study_id=phs000357 The Scleroderma Family Registry and DNA Repository (Registry) was initially developed as a registry and bio-specimen repository of patients with systemic sclerosis (scleroderma), family members and unaffected healthy controls. A case-control design was later adopted due to the lack of availability of many parents in this adult-onset disease, which precluded a linkage approach. In addition to collecting demographic data, the registry included the collection of disease-pertinent, cross-sectional, clinical information from medical records of affected participants. Registry participants were /= 18 years of age at enrollment. On the basis of medical record review, all cases were verified by the principal investigator to meet the eligibility criteria. 800+ blood samples from verified affected *cases* of European ancestry were selected for a Genome Wide Association Study (GWAS). These genetic data, as well as phenotypic data, are available in dbGaP. DNA, serum and plasma from Registry participants, which are currently being stored in the University of Texas Health Science Center - Houston, Scleroderma Registry Genetic Repository, are available for research purposes. However, not all of the 800+ registry participants who submitted a blood sample currently have DNA available due to depletion of some samples. In addition to including SNP data from the genome-wide (first study release) and immunochip (*second* study release) scan on the 800+ samples, the dbGaP database provides a set of data tables with phenotypic information collected from the participants in the registry.

Link

dbGaP study = phs000357

Trefwoorden

  1. 04-09-22 04-09-22 - Simon Heim
  2. 12-10-22 12-10-22 - Adrian Schulz
Houder van rechten

Maureen D. Mayes, MD, MPH, University of Texas Health Science Center, Houston, TX, USA

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12 oktober 2022

DOI

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Licentie

Creative Commons BY 4.0

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dbGaP phs000357 Genome-Wide Association Study in Systemic Sclerosis

Eligibility Criteria

Inclusion and exclusion criteria
Beschrijving

Inclusion and exclusion criteria

Individuals are considered eligible if the diagnosis of systemic sclerosis is confirmed by meeting the 1980 Preliminary Criteria for the Classification of Systemic Sclerosis OR if 3 of the 5 CREST features are documented to be present by medical record review as outlined below.
Beschrijving

Individuals are considered eligible if the diagnosis of systemic sclerosis is confirmed by meeting the 1980 Preliminary Criteria for the Classification of Systemic Sclerosis OR if 3 of the 5 CREST features are documented to be present by medical record review as outlined below.

Datatype

boolean

Alias
UMLS CUI [1,1]
C1706256
UMLS CUI [1,2]
C0036421
UMLS CUI [1,3]
C0679228
*Inclusion Criteria: 1980 Preliminary Criteria for the Classification of Systemic Sclerosis*
Beschrijving

*Inclusion Criteria: 1980 Preliminary Criteria for the Classification of Systemic Sclerosis*

Datatype

boolean

Alias
UMLS CUI [1,1]
C1706256
UMLS CUI [1,2]
C1512693
UMLS CUI [1,3]
C0679228
UMLS CUI [1,4]
C0036421
The American College of Rheumatology (former American Rheumatism Association - ARA) has defined criteria, that are 97% sensitive and 98% specific for systemic sclerosis (SSc) as follows:
Beschrijving

The American College of Rheumatology (former American Rheumatism Association - ARA) has defined criteria, that are 97% sensitive and 98% specific for systemic sclerosis (SSc) as follows:

Datatype

boolean

Alias
UMLS CUI [1,1]
C1706256
UMLS CUI [1,2]
C1512693
UMLS CUI [1,3]
C0035452
UMLS CUI [1,4]
C0679228
*Major criterion:*
Beschrijving

*Major criterion:*

Datatype

boolean

Alias
UMLS CUI [1,1]
C1706256
UMLS CUI [1,2]
C1512693
UMLS CUI [1,3]
C0205164
UMLS CUI [1,4]
C0243161
Skin thickening (skin sclerosis) involving the fingers and extending proximal to the metacarpal phalangeal (MCP) joints.
Beschrijving

Skin thickening (skin sclerosis) involving the fingers and extending proximal to the metacarpal phalangeal (MCP) joints.

Datatype

boolean

Alias
UMLS CUI [1,1]
C1706256
UMLS CUI [1,2]
C1512693
UMLS CUI [1,3]
C0205164
UMLS CUI [1,4]
C0243161
UMLS CUI [1,5]
C0241165
UMLS CUI [1,6]
C0205107
UMLS CUI [1,7]
C0025525
*Minor criteria:*
Beschrijving

*Minor criteria:*

Datatype

boolean

Alias
UMLS CUI [1,1]
C1706256
UMLS CUI [1,2]
C1512693
UMLS CUI [1,3]
C0205165
UMLS CUI [1,4]
C0243161
Sclerodactyly (skin thickening confined to the fingers and/or toes)
Beschrijving

Sclerodactyly (skin thickening confined to the fingers and/or toes)

Datatype

boolean

Alias
UMLS CUI [1,1]
C1706256
UMLS CUI [1,2]
C1512693
UMLS CUI [1,3]
C0205165
UMLS CUI [1,4]
C0243161
UMLS CUI [1,5]
C0150988
Digital pitting scars or loss of substance of the digital finger pads (pulp loss)
Beschrijving

Digital pitting scars or loss of substance of the digital finger pads (pulp loss)

Datatype

boolean

Alias
UMLS CUI [1,1]
C1706256
UMLS CUI [1,2]
C1512693
UMLS CUI [1,3]
C0205165
UMLS CUI [1,4]
C0243161
UMLS CUI [1,5]
C3854542
UMLS CUI [1,6]
C1410898
UMLS CUI [1,7]
C0459764
Bilateral basilar pulmonary fibrosis
Beschrijving

Bilateral basilar pulmonary fibrosis

Datatype

boolean

Alias
UMLS CUI [1,1]
C1706256
UMLS CUI [1,2]
C1512693
UMLS CUI [1,3]
C0205165
UMLS CUI [1,4]
C0243161
UMLS CUI [1,5]
C0034069
UMLS CUI [1,6]
C0238767
UMLS CUI [1,7]
C1184146
The patient should fulfill the major criterion or two of the three minor criteria.
Beschrijving

The patient should fulfill the major criterion or two of the three minor criteria.

Datatype

boolean

Alias
UMLS CUI [1,1]
C1706256
UMLS CUI [1,2]
C1512693
UMLS CUI [1,3]
C0243161
*Subsets of Systemic Sclerosis* <th></th> <th>Diffuse</th> <th>Limited*</th> Skin involvement Distal and proximal Extremities and/or the trunk Distal to elbows and knees
Beschrijving

*Subsets of Systemic Sclerosis* Skin involvement Distal and proximal Extremities and/or the trunk Distal to elbows and knees

Datatype

boolean

Alias
UMLS CUI [1,1]
C1512693
UMLS CUI [1,2]
C1706256
UMLS CUI [1,3]
C0036421
UMLS CUI [1,4]
C1515010
In addition, a diagnosis of systemic sclerosis is considered present if at least 3 out of 5 of the CREST features are present. These include: Calcinosis, Raynaud's phenomenon, esophageal dysmotility, Sclerodactyly and Telangiectasias.
Beschrijving

In addition, a diagnosis of systemic sclerosis is considered present if at least 3 out of 5 of the CREST features are present. These include: Calcinosis, Raynaud's phenomenon, esophageal dysmotility, Sclerodactyly and Telangiectasias.

Datatype

boolean

Alias
UMLS CUI [1,1]
C1512693
UMLS CUI [1,2]
C1706256
UMLS CUI [1,3]
C0036421
UMLS CUI [1,4]
C0206138
UMLS CUI [1,5]
C0006663
UMLS CUI [1,6]
C0034735
UMLS CUI [1,7]
C0014858
UMLS CUI [1,8]
C0150988
UMLS CUI [1,9]
C0039446
*Exclusion Criteria:*
Beschrijving

*Exclusion Criteria:*

Datatype

boolean

Alias
UMLS CUI [1,1]
C0680251
UMLS CUI [1,2]
C1706256
<18 years of age
Beschrijving

<18 years of age

Datatype

boolean

Alias
UMLS CUI [1,1]
C0680251
UMLS CUI [1,2]
C1706256
UMLS CUI [1,3]
C0001779
Unable to provide consent
Beschrijving

Unable to provide consent

Datatype

boolean

Alias
UMLS CUI [1,1]
C0680251
UMLS CUI [1,2]
C1706256
UMLS CUI [1,3]
C0021430
UMLS CUI [1,4]
C1299582

Similar models

Eligibility Criteria

Name
Type
Description | Question | Decode (Coded Value)
Datatype
Alias
Item Group
Inclusion and exclusion criteria
Individuals are considered eligible if the diagnosis of systemic sclerosis is confirmed by meeting the 1980 Preliminary Criteria for the Classification of Systemic Sclerosis OR if 3 of the 5 CREST features are documented to be present by medical record review as outlined below.
Item
Individuals are considered eligible if the diagnosis of systemic sclerosis is confirmed by meeting the 1980 Preliminary Criteria for the Classification of Systemic Sclerosis OR if 3 of the 5 CREST features are documented to be present by medical record review as outlined below.
boolean
C1706256 (UMLS CUI [1,1])
C0036421 (UMLS CUI [1,2])
C0679228 (UMLS CUI [1,3])
*Inclusion Criteria: 1980 Preliminary Criteria for the Classification of Systemic Sclerosis*
Item
*Inclusion Criteria: 1980 Preliminary Criteria for the Classification of Systemic Sclerosis*
boolean
C1706256 (UMLS CUI [1,1])
C1512693 (UMLS CUI [1,2])
C0679228 (UMLS CUI [1,3])
C0036421 (UMLS CUI [1,4])
The American College of Rheumatology (former American Rheumatism Association - ARA) has defined criteria, that are 97% sensitive and 98% specific for systemic sclerosis (SSc) as follows:
Item
The American College of Rheumatology (former American Rheumatism Association - ARA) has defined criteria, that are 97% sensitive and 98% specific for systemic sclerosis (SSc) as follows:
boolean
C1706256 (UMLS CUI [1,1])
C1512693 (UMLS CUI [1,2])
C0035452 (UMLS CUI [1,3])
C0679228 (UMLS CUI [1,4])
*Major criterion:*
Item
*Major criterion:*
boolean
C1706256 (UMLS CUI [1,1])
C1512693 (UMLS CUI [1,2])
C0205164 (UMLS CUI [1,3])
C0243161 (UMLS CUI [1,4])
Skin thickening (skin sclerosis) involving the fingers and extending proximal to the metacarpal phalangeal (MCP) joints.
Item
Skin thickening (skin sclerosis) involving the fingers and extending proximal to the metacarpal phalangeal (MCP) joints.
boolean
C1706256 (UMLS CUI [1,1])
C1512693 (UMLS CUI [1,2])
C0205164 (UMLS CUI [1,3])
C0243161 (UMLS CUI [1,4])
C0241165 (UMLS CUI [1,5])
C0205107 (UMLS CUI [1,6])
C0025525 (UMLS CUI [1,7])
*Minor criteria:*
Item
*Minor criteria:*
boolean
C1706256 (UMLS CUI [1,1])
C1512693 (UMLS CUI [1,2])
C0205165 (UMLS CUI [1,3])
C0243161 (UMLS CUI [1,4])
Sclerodactyly (skin thickening confined to the fingers and/or toes)
Item
Sclerodactyly (skin thickening confined to the fingers and/or toes)
boolean
C1706256 (UMLS CUI [1,1])
C1512693 (UMLS CUI [1,2])
C0205165 (UMLS CUI [1,3])
C0243161 (UMLS CUI [1,4])
C0150988 (UMLS CUI [1,5])
Digital pitting scars or loss of substance of the digital finger pads (pulp loss)
Item
Digital pitting scars or loss of substance of the digital finger pads (pulp loss)
boolean
C1706256 (UMLS CUI [1,1])
C1512693 (UMLS CUI [1,2])
C0205165 (UMLS CUI [1,3])
C0243161 (UMLS CUI [1,4])
C3854542 (UMLS CUI [1,5])
C1410898 (UMLS CUI [1,6])
C0459764 (UMLS CUI [1,7])
Bilateral basilar pulmonary fibrosis
Item
Bilateral basilar pulmonary fibrosis
boolean
C1706256 (UMLS CUI [1,1])
C1512693 (UMLS CUI [1,2])
C0205165 (UMLS CUI [1,3])
C0243161 (UMLS CUI [1,4])
C0034069 (UMLS CUI [1,5])
C0238767 (UMLS CUI [1,6])
C1184146 (UMLS CUI [1,7])
The patient should fulfill the major criterion or two of the three minor criteria.
Item
The patient should fulfill the major criterion or two of the three minor criteria.
boolean
C1706256 (UMLS CUI [1,1])
C1512693 (UMLS CUI [1,2])
C0243161 (UMLS CUI [1,3])
*Subsets of Systemic Sclerosis* Skin involvement Distal and proximal Extremities and/or the trunk Distal to elbows and knees
Item
*Subsets of Systemic Sclerosis* <th></th> <th>Diffuse</th> <th>Limited*</th> Skin involvement Distal and proximal Extremities and/or the trunk Distal to elbows and knees
boolean
C1512693 (UMLS CUI [1,1])
C1706256 (UMLS CUI [1,2])
C0036421 (UMLS CUI [1,3])
C1515010 (UMLS CUI [1,4])
In addition, a diagnosis of systemic sclerosis is considered present if at least 3 out of 5 of the CREST features are present. These include: Calcinosis, Raynaud's phenomenon, esophageal dysmotility, Sclerodactyly and Telangiectasias.
Item
In addition, a diagnosis of systemic sclerosis is considered present if at least 3 out of 5 of the CREST features are present. These include: Calcinosis, Raynaud's phenomenon, esophageal dysmotility, Sclerodactyly and Telangiectasias.
boolean
C1512693 (UMLS CUI [1,1])
C1706256 (UMLS CUI [1,2])
C0036421 (UMLS CUI [1,3])
C0206138 (UMLS CUI [1,4])
C0006663 (UMLS CUI [1,5])
C0034735 (UMLS CUI [1,6])
C0014858 (UMLS CUI [1,7])
C0150988 (UMLS CUI [1,8])
C0039446 (UMLS CUI [1,9])
*Exclusion Criteria:*
Item
*Exclusion Criteria:*
boolean
C0680251 (UMLS CUI [1,1])
C1706256 (UMLS CUI [1,2])
<18 years of age
Item
<18 years of age
boolean
C0680251 (UMLS CUI [1,1])
C1706256 (UMLS CUI [1,2])
C0001779 (UMLS CUI [1,3])
Unable to provide consent
Item
Unable to provide consent
boolean
C0680251 (UMLS CUI [1,1])
C1706256 (UMLS CUI [1,2])
C0021430 (UMLS CUI [1,3])
C1299582 (UMLS CUI [1,4])

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