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

Birt-Hogg-Dube Syndrome Clinical Trial

Official title:

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

Clinical Trial Details — Status: Completed

Administrative data

• NCT number: NCT03040115
• Other study ID #: RLDC5714A
• Secondary ID: U54HL127672
• Status: Completed
• Start date: August 2015
• Est. completion date: August 2019

Study information

• Verified date: January 2024
• Source: University of Cincinnati
• Contact: n/a
• Is FDA regulated: No
• Study type: Observational [Patient Registry]

Clinical Trial 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.

Recruitment information / eligibility

• Status: Completed
• Enrollment: 104
• Est. completion date: August 2019
• Est. primary completion date: August 2019
• Accepts healthy volunteers: No
• Gender: All
• Age group: 18 Years and older

Eligibility

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

Related Conditions & MeSH terms

• Birt-Hogg-Dube Syndrome
• Syndrome

Intervention

Other:
• Non-Interventional Study

Locations

Country	Name	City	State
United States	University of Cincinnati	Cincinnati	Ohio

Sponsors (3)

Lead Sponsor	Collaborator
University of Cincinnati	National Heart, Lung, and Blood Institute (NHLBI), Rare Diseases Clinical Research Network

Country where clinical trial is conducted

United States, 

Outcome

Type	Measure	Description	Time frame	Safety issue
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.	During air travel or within 24 hours following air travel
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.	3 years
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).	3 years