Assessment of Safety of Air Travel in Patients With Birt-Hogg-Dube Syndrome

Completed

• Conditions: Birt-Hogg-Dube Syndrome

• Registration Number: NCT03040115
• Lead Sponsor: University of Cincinnati

Brief Summary

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.

Detailed Description

Not available

Study Timeline

• Study Start: 2015-08
• Study First Submitted: 2017-01-05
• Study First Submitted QC: 2017-01-30
• Study First Posted: 2017-02-02
• Primary Completion: 2019-08
• Study Completion: 2019-08
• Results First Submitted: 2023-01-14
• Status Verified: 2024-01
• Results First Submitted QC: 2024-01-03
• Last Update Submitted: 2024-01-03
• Results First Posted: 2024-01-31
• Last Update Posted: 2024-01-31

Recruitment & Eligibility

• Status: COMPLETED
• Sex: All
• Target Recruitment: 104

Inclusion Criteria

• Adult, age 18 or older
• English literate
• Signed, dated informed consent; either given electronically or via paper form
• Diagnosis of BHD confirmed by either a) the presence of fibrofolliculomas/trichodiscomas on skin biopsy, or b) the presence of pathogenic FLCN mutations.

Exclusion Criteria

• Inability to give informed consent

Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
Number of Patients With Birt-Hogg-Dube Syndrome Who Experienced Spontaneous Pneumothorax During or Within 24 Hours Following Air Travel	During air travel 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.

Secondary Outcome Measures

Name	Time	Method
Number of Patients With Birt-Hogg-Dube Syndrome Who Experience Recurrent Pneumothorax Following Pleurodesis	3 years	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).
Number of Patients With Birt-Hogg-Dube Syndrome Who Experience a Spontaneous Pneumothorax	3 years	The investigators will catalogue the number of spontaneous pneumothoraces experienced by patients with Birt-Hogg-Dube syndrome.

Trial Locations

Locations (1)

University of Cincinnati; 🇺🇸 Cincinnati, Ohio, United States