Symptoms and Treatment Results in Hereditary Hemorrhagic Telangiectasia

March 21, 2022 updated by: Urban Geisthoff, University Hospital, Essen

A Questionnaire Based Study on Symptoms and Treatment Results in Hereditary Hemorrhagic Telangiectasia (HHT)

Hereditary haemorrhagic telangiectasia (HHT), also known as Rendu-Osler-Weber syndrome, is an inherited multisystemic disorder with recurrent epistaxis, mucocutaneous telangiectasia and visceral arteriovenous malformations. The purpose of this study is to provide data about multiple clinical aspects of HHT and responses to treatment. For comparison of some aspects also data of non-affected relatives is collected (second cohort). the questionnaire has been designed primarily for web based entry, but can also be circulated in paper format on request.

Study Overview

Status

Completed

Conditions

Intervention / Treatment

Detailed Description

Specific aspects include potential consequences from iron deficiency, efficacy and safety of self-packing, effect of female sex hormones, mortality, effects on the immune system.

Study Type

Observational

Enrollment (Actual)

915

Contacts and Locations

This section provides the contact details for those conducting the study, and information on where this study is being conducted.

Study Locations

  • Germany
      • Essen, Germany, 45147
        • University Hospital Essen

Participation Criteria

Researchers look for people who fit a certain description, called eligibility criteria. Some examples of these criteria are a person's general health condition or prior treatments.

Eligibility Criteria

Ages Eligible for Study

18 years and older (Adult, Older Adult)

Accepts Healthy Volunteers

No

Genders Eligible for Study

All

Sampling Method

Non-Probability Sample

Study Population

Patients with HHT; Indirectly we accept healthy volunteers only as control cohort in questionnaires of affected persons.

Description

Inclusion Criteria:

  • a diagnosis of Hereditary Haemorrhagic Telangiectasia

Exclusion Criteria:

  • unable to provide informed consent

Study Plan

This section provides details of the study plan, including how the study is designed and what the study is measuring.

How is the study designed?

Design Details

  • Observational Models: Cohort
  • Time Perspectives: Prospective

Cohorts and Interventions

Group / Cohort
Intervention / Treatment
patients with HHT
patients with Hereditary Haemorrhagic Telangiectasia
Other: questionnaire
a questionnaire based study
control cohort
indirectly only as a control cohort in questionnaire of affected persons

What is the study measuring?

Primary Outcome Measures

Outcome Measure
Measure Description
Time Frame
Effect of nasal self-packing to treat epistaxis on the quality of life in patients with HHT measured mainly by Glasgow Benefit Inventory (GBI)
Time Frame: 34 months
Hereditary Haemorrhagic Telangiectasia is characterised by recurrent epistaxis which can lead to a feeling to lose control. The investigators examine whether the use of high volume low pressure nasal packing is a secure and practical method to improve patients' quality of life.
34 months

Secondary Outcome Measures

Outcome Measure
Time Frame
Number of participants with treatment-related adverse events as assessed by CTCAE v4.0
Time Frame: 34 months
34 months

Other Outcome Measures

Outcome Measure
Time Frame
Number of patients with restless legs syndrome according to a questionnaire including the criteria by Allen et al., Sleep Medicine 4(2003) 101-119
Time Frame: 34 months
34 months
Number of infectious diseases and complications in comparison to healthy spouses
Time Frame: 34 months
34 months
Hormonal changes: Change of number of visible telangiectases during menstrual cycle and pregnancy
Time Frame: 34 months
34 months

Collaborators and Investigators

This is where you will find people and organizations involved with this study.

Sponsor

University Hospital, Essen

Investigators

  • Study Director: Urban Geisthoff, Prof. Dr., University Hospital, Essen

Publications and helpful links

The person responsible for entering information about the study voluntarily provides these publications. These may be about anything related to the study.

Helpful Links

Study record dates

These dates track the progress of study record and summary results submissions to ClinicalTrials.gov. Study records and reported results are reviewed by the National Library of Medicine (NLM) to make sure they meet specific quality control standards before being posted on the public website.

Study Major Dates

Study Start (Actual)

April 1, 2014

Primary Completion (Actual)

August 1, 2019

Study Completion (Actual)

March 1, 2021

Study Registration Dates

First Submitted

February 9, 2016

First Submitted That Met QC Criteria

February 19, 2016

First Posted (Estimate)

February 24, 2016

Study Record Updates

Last Update Posted (Actual)

March 22, 2022

Last Update Submitted That Met QC Criteria

March 21, 2022

Last Verified

March 1, 2022

More Information

Terms related to this study

Additional Relevant MeSH Terms

Other Study ID Numbers

  • University Clinic Essen

Plan for Individual participant data (IPD)

Plan to Share Individual Participant Data (IPD)?

Undecided

This information was retrieved directly from the website clinicaltrials.gov without any changes. If you have any requests to change, remove or update your study details, please contact register@clinicaltrials.gov. As soon as a change is implemented on clinicaltrials.gov, this will be updated automatically on our website as well.

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