Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
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.
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.
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
| Label | Type | Description | Intervention Names |
|---|---|---|---|
| patients with HHT | patients with Hereditary Haemorrhagic Telangiectasia |
| |
| control cohort | indirectly only as a control cohort in questionnaire of affected persons |
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| questionnaire | Other | a questionnaire based study |
|
| 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) | 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 |
| Measure | Description | Time Frame |
|---|---|---|
| Number of participants with treatment-related adverse events as assessed by CTCAE v4.0 | 34 months |
| Measure | Description | 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 | 34 months | |
| Number of infectious diseases and complications in comparison to healthy spouses | 34 months |
Inclusion Criteria:
Exclusion Criteria:
Not provided
Not provided
Not provided
Patients with HHT; Indirectly we accept healthy volunteers only as control cohort in questionnaires of affected persons.
Not provided
| Name | Affiliation | Role |
|---|---|---|
| Urban Geisthoff, Prof. Dr. | University Hospital, Essen | Study Director |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| University Hospital Essen | Essen | 45147 | Germany |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 41862858 | Derived | Droege F, Thangavelu K, Koeninger A, Huessler EM, Lang S, Stuck BA, Geisthoff UW. Influence of hormonal status in hereditary hemorrhagic telangiectasia - analysis of an online patient questionnaire. BMC Pregnancy Childbirth. 2026 Mar 20;26(1):388. doi: 10.1186/s12884-026-08928-2. |
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
| ID | Term |
|---|---|
| D013683 | Telangiectasia, Hereditary Hemorrhagic |
| ID | Term |
|---|---|
| D020141 | Hemostatic Disorders |
| D014652 | Vascular Diseases |
| D002318 | Cardiovascular Diseases |
| D013684 | Telangiectasis |
Not provided
Not provided
| ID | Term |
|---|---|
| D011795 | Surveys and Questionnaires |
| ID | Term |
|---|---|
| D003625 | Data Collection |
| D004812 | Epidemiologic Methods |
| D008919 | Investigative Techniques |
| D017531 | Health Care Evaluation Mechanisms |
Not provided
Not provided
Not provided
Not provided
Not provided
| Hormonal changes: Change of number of visible telangiectases during menstrual cycle and pregnancy | 34 months |
| D006474 |
| Hemorrhagic Disorders |
| D006402 | Hematologic Diseases |
| D006425 | Hemic and Lymphatic Diseases |
| D054079 | Vascular Malformations |
| D018376 | Cardiovascular Abnormalities |
| D000013 | Congenital Abnormalities |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D011787 | Quality of Health Care |
| D017530 | Health Care Quality, Access, and Evaluation |
| D011634 | Public Health |
| D004778 | Environment and Public Health |