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ID

46171

Description

Principal Investigator: Garry R. Cutting, MD, Johns Hopkins Medical Institutes, McKusick-Nathans Institute of Genetic Medicine, Baltimore, MD, USA MeSH: Cystic Fibrosis https://www.ncbi.nlm.nih.gov/projects/gap/cgi-bin/study.cgi?study_id=phs000556 The NHLBI "Grand Opportunity" Exome Sequencing Project (GO-ESP), a signature project of the NHLBI Recovery Act investment, was designed to identify genetic variants in coding regions (exons) of the human genome (the "exome") that are associated with heart, lung and blood diseases. These and related diseases that are of high impact to public health and individuals from diverse racial and ethnic groups will be studied. These data may help researchers understand the causes of disease, contributing to better ways to prevent, diagnose, and treat diseases, as well as determine whether to tailor prevention and treatments to specific populations. This could lead to more effective treatments and reduce the likelihood of side effects. GO-ESP is comprised of five collaborative components: 3 cohort consortia - HeartGO, LungGO, and WHISP - and 2 sequencing centers - BroadGO and SeattleGO. Cystic fibrosis (CF) patients without detectable disease-causing mutations in CFTR present a clinical phenotype very similar to, but possibly discrete from, classic CF. We hypothesize that the phenotype of these atypical CF patients is not due only to mutations in the carbonic anhydrase gene CA12 or in the genes encoding the epithelium sodium ion channel ENaC, but is due to mutations in novel genes that have yet to be associated with CF. The goal of this study is to determine the molecular etiology of this atypical CF.

Link

https://www.ncbi.nlm.nih.gov/projects/gap/cgi-bin/study.cgi?study_id=phs000556

Keywords

  1. 11/2/22 11/2/22 - Simon Heim
  2. 1/29/25 1/29/25 - Akane Nishihara
Copyright Holder

Garry R. Cutting, MD, Johns Hopkins Medical Institutes, McKusick-Nathans Institute of Genetic Medicine, Baltimore, MD, USA

Uploaded on

January 29, 2025

DOI

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License

Creative Commons BY 4.0

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    dbGaP phs000556 NHLBI GO-ESP: Family Studies (Atypical Cystic Fibrosis)

    Subject ID, age, sex, race, and current status of subjects affected or not affected with cystic fibrosis and involved in the "Johns Hopkins Medical Institutes (JHMI) Molecular Genetics of Atypical Cystic Fibrosis (CF) Study" project.

    pht003139
    Description

    pht003139

    De-identified subject ID
    Description

    SUBJID

    Data type

    text

    Alias
    UMLS CUI [1,1]
    C4684638
    UMLS CUI [1,2]
    C2348585
    Average sweat chloride Levels
    Description

    Chloride

    Data type

    text

    Measurement units
    • mM Cl-
    Alias
    UMLS CUI [1,1]
    C1304760
    UMLS CUI [1,2]
    C1510992
    mM Cl-
    Forced expiratory volume
    Description

    FEV1

    Data type

    text

    Measurement units
    • %
    Alias
    UMLS CUI [1,1]
    C0016529
    %
    Pancreatic status
    Description

    PS

    Data type

    text

    Alias
    UMLS CUI [1,1]
    C0232786
    Gender of participant
    Description

    Sex

    Data type

    text

    Alias
    UMLS CUI [1,1]
    C0079399
    Ethnic origins of patient [Asian, European, Middle-Eastern, Other, Unknown]
    Description

    Ethnicity

    Data type

    string

    Alias
    UMLS CUI [1,1]
    C0015031
    Lung infections [None, Staph, P. aeruginosa, Aspergillus, MRSA, H. influenzae, Strep, Other]
    Description

    LungInf

    Data type

    string

    Alias
    UMLS CUI [1,1]
    C0876973
    Additional clinical presentations
    Description

    OtherClinical

    Data type

    string

    Alias
    UMLS CUI [1,1]
    C2707270

    Similar models

    Subject ID, age, sex, race, and current status of subjects affected or not affected with cystic fibrosis and involved in the "Johns Hopkins Medical Institutes (JHMI) Molecular Genetics of Atypical Cystic Fibrosis (CF) Study" project.

    Name
    Type
    Description | Question | Decode (Coded Value)
    Data type
    Alias
    Item Group
    pht003139
    SUBJID
    Item
    De-identified subject ID
    text
    C4684638 (UMLS CUI [1,1])
    C2348585 (UMLS CUI [1,2])
    Chloride
    Item
    Average sweat chloride Levels
    text
    C1304760 (UMLS CUI [1,1])
    C1510992 (UMLS CUI [1,2])
    FEV1
    Item
    Forced expiratory volume
    text
    C0016529 (UMLS CUI [1,1])
    Item
    Pancreatic status
    text
    C0232786 (UMLS CUI [1,1])
    Code List
    Pancreatic status
    CL Item
    Pancreatic insufficient (PI)
    CL Item
    Pancreatic sufficient (PS)
    Item
    Gender of participant
    text
    C0079399 (UMLS CUI [1,1])
    Code List
    Gender of participant
    CL Item
    Female (F)
    CL Item
    Male (M)
    CL Item
    Not applicable (NA)
    CL Item
    Unknown (UNK)
    Ethnicity
    Item
    Ethnic origins of patient [Asian, European, Middle-Eastern, Other, Unknown]
    string
    C0015031 (UMLS CUI [1,1])
    LungInf
    Item
    Lung infections [None, Staph, P. aeruginosa, Aspergillus, MRSA, H. influenzae, Strep, Other]
    string
    C0876973 (UMLS CUI [1,1])
    OtherClinical
    Item
    Additional clinical presentations
    string
    C2707270 (UMLS CUI [1,1])

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