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| ID | Type | Description | Link |
|---|---|---|---|
| U54HL127672 | U.S. NIH Grant/Contract | View source |
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| Name | Class |
|---|---|
| Rare Diseases Clinical Research Network | NETWORK |
| National Heart, Lung, and Blood Institute (NHLBI) | NIH |
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The aim of this study is to conduct survey-based assessments for the safety of air travel in patients with Birt-Hogg-Dube syndrome (BHD). The study will enroll patients through the clinic network at Rare Lung Disease Consortium (RLDC) and through the BHD foundation website. Patients will have access to the questionnaire via REDCap (an online data management system) and each patient will be provided with a link to complete the survey. The investigators plan on enrolling approximately 100 patients with BHD for the purpose of this study. Secondary aims of this study include further characterization of the clinical aspects of disease and to establish a contact registry for these patients, in order to facilitate future studies.
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| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Non-Interventional Study | Other |
| Measure | Description | Time Frame |
|---|---|---|
| Number of Patients With Birt-Hogg-Dube Syndrome Who Experienced Spontaneous Pneumothorax During or Within 24 Hours Following Air Travel | The investigators will measure the number of pneumothoraces that occur either during air travel, or within 24 hours of air travel among patients with Birt-Hogg-Dube syndrome. The incidence of air travel related pneumothorax will be calculated by dividing the number of pneumothoraces to the total number of flights undertaken by the entire cohort. | During air travel or within 24 hours following air travel |
| Measure | Description | Time Frame |
|---|---|---|
| Number of Patients With Birt-Hogg-Dube Syndrome Who Experience a Spontaneous Pneumothorax | The investigators will catalogue the number of spontaneous pneumothoraces experienced by patients with Birt-Hogg-Dube syndrome. | 3 years |
| Number of Patients With Birt-Hogg-Dube Syndrome Who Experience Recurrent Pneumothorax Following Pleurodesis |
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Inclusion Criteria:
Exclusion Criteria:
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All patients with a confirmed diagnosis of Birt-Hogg-Dube syndrome are eligible to participate in this study.
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| Name | Affiliation | Role |
|---|---|---|
| Nishant Gupta, MD | University of Cincinnati | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| University of Cincinnati | Cincinnati | Ohio | 45267 | United States |
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| ID | Title | Description |
|---|---|---|
| FG000 | Patients Self-reporting With BHD | Patients self-reporting with BHD |
| Title | Milestones | Reasons Not Completed | ||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Overall Study |
|
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| ID | Title | Description |
|---|---|---|
| BG000 | Patients Self-reporting With BHD | Patients self-reporting with BHD |
| Units | Counts |
|---|---|
| Participants |
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| Title | Description | Population Description | Parameter Type | Dispersion Type | Unit of Measure | Calculate Percentage | Denominator Units Selected | Denominators | Classes |
|---|---|---|---|---|---|---|---|---|---|
| Age, Continuous | Age at time of survey collection |
| Type | Title | Description | Population Description | Reporting Status | Anticipated Posting Date | Parameter Type | Dispersion Type | Unit of Measure | Calculate Percentage | Time Frame | Units Analyzed | Denominator Units Selected | Arm/Group Information | Denominators | Classes | Analyses | ||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Primary | Number of Patients With Birt-Hogg-Dube Syndrome Who Experienced Spontaneous Pneumothorax During or Within 24 Hours Following Air Travel | The investigators will measure the number of pneumothoraces that occur either during air travel, or within 24 hours of air travel among patients with Birt-Hogg-Dube syndrome. The incidence of air travel related pneumothorax will be calculated by dividing the number of pneumothoraces to the total number of flights undertaken by the entire cohort. | Posted | Count of Participants | Participants | During air travel or within 24 hours following air travel |
|
Not applicable. Cross sectional study.
Cross sectional study. No data regarding mortality, serious or other adverse events was collected as part of the study.
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| ID | Title | Description | Deaths (Affected) | Deaths (At Risk) | Serious Events (Affected) | Serious Events (At Risk) | Other Events (Affected) | Other Events (At Risk) |
|---|---|---|---|---|---|---|---|---|
| EG000 | AEs were not collected as part of this observational study. | 0 |
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| Title | Organization | Phone | Extension | |
|---|---|---|---|---|
| Elizabeth Kopras, Senior Research Associate | University of Cincinnati | 513-558-7205 | koprasej@uc.edu |
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| Type | Includes Protocol | Includes SAP | Includes ICF | Document Label | Document Date | Document Uploaded Date | Document File Name |
|---|---|---|---|---|---|---|---|
| Prot | Yes | No | No | Study Protocol | Feb 23, 2017 | Dec 8, 2023 | Prot_000.pdf |
| SAP | No | Yes | No | Statistical Analysis Plan | Aug 5, 2015 | Sep 18, 2023 | SAP_001.pdf |
| ICF | No | No | Yes | Informed Consent Form | Feb 23, 2017 | Dec 8, 2023 | ICF_002.pdf |
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| ID | Term |
|---|---|
| D058249 | Birt-Hogg-Dube Syndrome |
| ID | Term |
|---|---|
| D009386 | Neoplastic Syndromes, Hereditary |
| D009369 | Neoplasms |
| D030342 | Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
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The investigators will measure the incidence of ipsilateral pneumothorax following pleurodesis in order to determine the efficacy (as measured by the rate of pneumothorax recurrence following pleurodesis). |
| 3 years |
| Median |
| Inter-Quartile Range |
| years |
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| Sex: Female, Male | Patients self-reported gender. | Count of Participants | Participants |
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| Race and Ethnicity Not Collected | Race and Ethnicity were not collected from any participant. | Count of Participants | Participants |
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| Region of Enrollment | Count of Participants | Participants |
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| Number of Participants with a History of Air Travel | Number | participants |
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| Units |
|---|
| Counts |
|---|
| Participants |
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| Secondary | Number of Patients With Birt-Hogg-Dube Syndrome Who Experience a Spontaneous Pneumothorax | The investigators will catalogue the number of spontaneous pneumothoraces experienced by patients with Birt-Hogg-Dube syndrome. | Posted | Count of Participants | Participants | 3 years |
|
|
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| Secondary | Number of Patients With Birt-Hogg-Dube Syndrome Who Experience Recurrent Pneumothorax Following Pleurodesis | The investigators will measure the incidence of ipsilateral pneumothorax following pleurodesis in order to determine the efficacy (as measured by the rate of pneumothorax recurrence following pleurodesis). | Posted | Count of Participants | Participants | 3 years |
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| 0 |
| 0 |
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| 0 |
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