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

NCT03040115 | Completed Results DMC

• 2 other identifiers: RLDC5714AU54HL127672
• Study Type: observational
• Target: 104
• Locations: 1 country
• Sites: 1

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.

Conditions

Birt-Hogg-Dube Syndrome

Outcome Measures

Primary Outcomes (1)

• 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 Outcomes (2)

• Number of Patients With Birt-Hogg-Dube Syndrome Who Experience a Spontaneous Pneumothorax

  3 years

• Number of Patients With Birt-Hogg-Dube Syndrome Who Experience Recurrent Pneumothorax Following Pleurodesis

  3 years

Interventions

Non-Interventional Study OTHER

Eligibility Criteria

• Age: 18 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

All patients with a confirmed diagnosis of Birt-Hogg-Dube syndrome are eligible to participate in this study.

You may qualify if:

• 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.

You may not qualify if:

• Inability to give informed consent

Sponsors & Collaborators

• University of Cincinnati: lead
• Rare Diseases Clinical Research Network: collaborator
• National Heart, Lung, and Blood Institute (NHLBI): collaborator

Study Sites (1)

University of Cincinnati

Cincinnati, Ohio, 45267, United States

Location

MeSH Terms

Conditions

Birt-Hogg-Dube Syndrome

Condition Hierarchy (Ancestors)

Neoplastic Syndromes, Hereditary; Neoplasms; Genetic Diseases, Inborn; Congenital, Hereditary, and Neonatal Diseases and Abnormalities

Results Point of Contact

• Title: Elizabeth Kopras, Senior Research Associate
• Organization: University of Cincinnati

koprasej@uc.edu

513-558-7205

Study Officials

• Nishant Gupta, MD

  University of Cincinnati

  PRINCIPAL INVESTIGATOR

Publication Agreements

• PI is Sponsor Employee: Yes

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: CROSS SECTIONAL
• Target Duration: 3 Years
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Adjunct Assistant Professor

Study Record Dates

• First Submitted: January 5, 2017
• First Posted: February 2, 2017
• Study Start: August 1, 2015
• Primary Completion: August 1, 2019
• Study Completion: August 1, 2019
• Last Updated: January 31, 2024
• Results First Posted: January 31, 2024

Record last verified: 2024-01

Locations

US (1)