<clinical_study>
  <!-- This xml conforms to an XML Schema at:
    https://clinicaltrials.gov/ct2/html/images/info/public.xsd -->
  <required_header>
    <download_date>ClinicalTrials.gov processed this data on September 20, 2023</download_date>
    <link_text>Link to the current ClinicalTrials.gov record.</link_text>
    <url>https://clinicaltrials.gov/ct2/show/NCT03040115</url>
  </required_header>
  <id_info>
    <org_study_id>RLDC5714A</org_study_id>
    <secondary_id>U54HL127672</secondary_id>
    <nct_id>NCT03040115</nct_id>
  </id_info>
  <brief_title>Assessment of Safety of Air Travel in Patients With Birt-Hogg-Dube Syndrome</brief_title>
  <official_title>Assessment of Safety of Air Travel in Patients With Birt-Hogg-Dube Syndrome</official_title>
  <sponsors>
    <lead_sponsor>
      <agency>University of Cincinnati</agency>
      <agency_class>Other</agency_class>
    </lead_sponsor>
    <collaborator>
      <agency>Rare Diseases Clinical Research Network</agency>
      <agency_class>Other</agency_class>
    </collaborator>
    <collaborator>
      <agency>National Heart, Lung, and Blood Institute (NHLBI)</agency>
      <agency_class>NIH</agency_class>
    </collaborator>
  </sponsors>
  <source>University of Cincinnati</source>
  <oversight_info>
    <has_dmc>Yes</has_dmc>
  </oversight_info>
  <brief_summary>
    <textblock>
      The aim of this study is to conduct survey-based assessments for the safety of air travel in&#xD;
      patients with Birt-Hogg-Dube syndrome (BHD). The study will enroll patients through the&#xD;
      clinic network at Rare Lung Disease Consortium (RLDC) and through the BHD foundation website.&#xD;
      Patients will have access to the questionnaire via REDCap (an online data management system)&#xD;
      and each patient will be provided with a link to complete the survey. The investigators plan&#xD;
      on enrolling approximately 100 patients with BHD for the purpose of this study. Secondary&#xD;
      aims of this study include further characterization of the clinical aspects of disease and to&#xD;
      establish a contact registry for these patients, in order to facilitate future studies.&#xD;
    </textblock>
  </brief_summary>
  <overall_status>Unknown status</overall_status>
  <last_known_status>Recruiting</last_known_status>
  <start_date>August 2015</start_date>
  <completion_date type="Anticipated">December 2018</completion_date>
  <primary_completion_date type="Anticipated">December 2018</primary_completion_date>
  <study_type>Observational [Patient Registry]</study_type>
  <has_expanded_access>No</has_expanded_access>
  <study_design_info>
    <observational_model>Cohort</observational_model>
    <time_perspective>Cross-Sectional</time_perspective>
  </study_design_info>
  <target_duration>3 Years</target_duration>
  <primary_outcome>
    <measure>Incidence of spontaneous pneumothorax during or within 24 hours following air travel</measure>
    <time_frame>3 years</time_frame>
    <description>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.</description>
  </primary_outcome>
  <secondary_outcome>
    <measure>Rate of recurrent pneumothorax in patients with Birt-Hogg-Dube syndrome</measure>
    <time_frame>3 years</time_frame>
    <description>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.</description>
  </secondary_outcome>
  <secondary_outcome>
    <measure>Efficacy of pleurodesis in reducing the risk of recurrent pneumothoraces in Birt-Hogg-Dube syndrome</measure>
    <time_frame>3 years</time_frame>
    <description>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.</description>
  </secondary_outcome>
  <enrollment type="Anticipated">100</enrollment>
  <condition>Birt-Hogg-Dube Syndrome</condition>
  <intervention>
    <intervention_type>Other</intervention_type>
    <intervention_name>Non-Interventional Study</intervention_name>
  </intervention>
  <eligibility>
    <study_pop>
      <textblock>
        All patients with a confirmed diagnosis of Birt-Hogg-Dube syndrome are eligible to&#xD;
        participate in this study.&#xD;
      </textblock>
    </study_pop>
    <sampling_method>Non-Probability Sample</sampling_method>
    <criteria>
      <textblock>
        Inclusion Criteria:&#xD;
&#xD;
          -  Adult, age 18 or older&#xD;
&#xD;
          -  English literate&#xD;
&#xD;
          -  Signed, dated informed consent; either given electronically or via paper form&#xD;
&#xD;
          -  Diagnosis of BHD confirmed by either a) the presence of&#xD;
             fibrofolliculomas/trichodiscomas on skin biopsy, or b) the presence of pathogenic FLCN&#xD;
             mutations.&#xD;
&#xD;
        Exclusion Criteria:&#xD;
&#xD;
          -  Inability to give informed consent&#xD;
      </textblock>
    </criteria>
    <gender>All</gender>
    <minimum_age>18 Years</minimum_age>
    <maximum_age>N/A</maximum_age>
    <healthy_volunteers>No</healthy_volunteers>
  </eligibility>
  <overall_contact>
    <last_name>Elizabeth J Kopras, BA</last_name>
    <phone>513-558-7205</phone>
    <email>koprasej@ucmail.uc.edu</email>
  </overall_contact>
  <location>
    <facility>
      <name>University of Cincinnati</name>
      <address>
        <city>Cincinnati</city>
        <state>Ohio</state>
        <zip>45267</zip>
        <country>United States</country>
      </address>
    </facility>
    <status>Recruiting</status>
    <contact>
      <last_name>Elizabeth J Kopras, BA</last_name>
      <phone>513-558-7205</phone>
      <email>koprasej@ucmail.uc.edu</email>
    </contact>
    <investigator>
      <last_name>Nishant Gupta, MD</last_name>
      <role>Principal Investigator</role>
    </investigator>
  </location>
  <location_countries>
    <country>United States</country>
  </location_countries>
  <link>
    <url>https://redcap.research.cchmc.org/surveys/?s=oNTxfZ9gqz</url>
    <description>Link to the study questionnaire</description>
  </link>
  <verification_date>January 2017</verification_date>
  <study_first_submitted>January 5, 2017</study_first_submitted>
  <study_first_submitted_qc>January 30, 2017</study_first_submitted_qc>
  <study_first_posted type="Estimate">February 2, 2017</study_first_posted>
  <last_update_submitted>January 30, 2017</last_update_submitted>
  <last_update_submitted_qc>January 30, 2017</last_update_submitted_qc>
  <last_update_posted type="Estimate">February 2, 2017</last_update_posted>
  <responsible_party>
    <responsible_party_type>Principal Investigator</responsible_party_type>
    <investigator_affiliation>University of Cincinnati</investigator_affiliation>
    <investigator_full_name>Nishant Gupta</investigator_full_name>
    <investigator_title>Adjunct Assistant Professor</investigator_title>
  </responsible_party>
  <condition_browse>
    <!-- CAUTION:  The following MeSH terms are assigned with an imperfect algorithm            -->
    <mesh_term>Birt-Hogg-Dube Syndrome</mesh_term>
    <mesh_term>Syndrome</mesh_term>
  </condition_browse>
  <pending_results>
    <submitted>January 14, 2023</submitted>
    <returned>February 10, 2023</returned>
  </pending_results>
  <!-- Results have not yet been posted for this study                                          -->
</clinical_study>


download date: ClinicalTrials.gov processed this data on September 20, 2023
link text: Link to the current ClinicalTrials.gov record.
url: https://clinicaltrials.gov/ct2/show/NCT03040115
org study id: RLDC5714A
secondary id: U54HL127672
nct id: NCT03040115
lead sponsor: 
collaborator: 
collaborator: 
has dmc: Yes
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.
observational model: Cohort
time perspective: Cross-Sectional
measure: Incidence of spontaneous pneumothorax during or within 24 hours following air travel
time frame: 3 years
description: 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.
measure: Rate of recurrent pneumothorax in patients with Birt-Hogg-Dube syndrome
time frame: 3 years
description: 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.
measure: Efficacy of pleurodesis in reducing the risk of recurrent pneumothoraces in Birt-Hogg-Dube syndrome
time frame: 3 years
description: 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.
intervention type: Other
intervention name: Non-Interventional Study
study pop: 
sampling method: Non-Probability Sample
criteria: 
gender: All
minimum age: 18 Years
maximum age: N/A
healthy volunteers: No
last name: Elizabeth J Kopras, BA
phone: 513-558-7205
email: koprasej@ucmail.uc.edu
facility: 
status: Recruiting
contact: 
investigator: 
country: United States
url: https://redcap.research.cchmc.org/surveys/?s=oNTxfZ9gqz
description: Link to the study questionnaire
responsible party type: Principal Investigator
investigator affiliation: University of Cincinnati
investigator full name: Nishant Gupta
investigator title: Adjunct Assistant Professor
mesh term: Birt-Hogg-Dube Syndrome
mesh term: Syndrome
submitted: January 14, 2023
returned: February 10, 2023