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

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ClinicalTrials.gov Identifier: NCT03040115
Recruitment Status : Recruiting
First Posted : February 2, 2017
Last Update Posted : February 2, 2017
Sponsor:
Collaborators:
Rare Diseases Clinical Research Network
National Heart, Lung, and Blood Institute (NHLBI)
Information provided by (Responsible Party):
Nishant Gupta, 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.

Condition or disease Intervention/treatment
Birt-Hogg-Dube Syndrome Other: Non-Interventional Study

Study Type : Observational [Patient Registry]
Estimated Enrollment : 100 participants
Observational Model: Cohort
Time Perspective: Cross-Sectional
Target Follow-Up Duration: 3 Years
Official Title: Assessment of Safety of Air Travel in Patients With Birt-Hogg-Dube Syndrome
Study Start Date : August 2015
Estimated Primary Completion Date : December 2018
Estimated Study Completion Date : December 2018





Primary Outcome Measures :
  1. Incidence of spontaneous pneumothorax during or within 24 hours following air travel [ Time Frame: 3 years ]
    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 :
  1. Rate of recurrent pneumothorax in patients with Birt-Hogg-Dube syndrome [ Time Frame: 3 years ]
    The investigators will catalogue the number roof spontaneous pneumothoraces experienced by patients with Birt-Hogg-Dube syndrome. These results will be used to calculate the proportion of patients who experience at least one recurrence of their pneumothorax, as well as the average number of pneumothoraces experienced per patient.

  2. Efficacy of pleurodesis in reducing the risk of recurrent pneumothoraces in Birt-Hogg-Dube syndrome [ Time Frame: 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). These results will be calculated separately for chemical and surgical pleurodesis.



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Ages Eligible for Study:   18 Years and older   (Adult, Older Adult)
Sexes Eligible for Study:   All
Accepts Healthy Volunteers:   No
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.
Criteria

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

Information from the National Library of Medicine

To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.

Please refer to this study by its ClinicalTrials.gov identifier (NCT number): NCT03040115


Contacts
Contact: Elizabeth J Kopras, BA 513-558-7205 koprasej@ucmail.uc.edu

Locations
United States, Ohio
University of Cincinnati Recruiting
Cincinnati, Ohio, United States, 45267
Contact: Elizabeth J Kopras, BA    513-558-7205    koprasej@ucmail.uc.edu   
Principal Investigator: Nishant Gupta, MD         
Sponsors and Collaborators
University of Cincinnati
Rare Diseases Clinical Research Network
National Heart, Lung, and Blood Institute (NHLBI)

Additional Information:
Responsible Party: Nishant Gupta, Adjunct Assistant Professor, University of Cincinnati
ClinicalTrials.gov Identifier: NCT03040115     History of Changes
Other Study ID Numbers: RLDC5714A
U54HL127672 ( U.S. NIH Grant/Contract )
First Posted: February 2, 2017    Key Record Dates
Last Update Posted: February 2, 2017
Last Verified: January 2017

Additional relevant MeSH terms:
Syndrome
Birt-Hogg-Dube Syndrome
Disease
Pathologic Processes
Neoplastic Syndromes, Hereditary
Neoplasms
Genetic Diseases, Inborn