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| Name | Class |
|---|---|
| DLR German Aerospace Center | OTHER |
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We examine patients with different autonomic neuropathies and Ehlers Danlos syndromes compared to healthy controls at three different points over time (baseline, after 3 months and after 1.5 years) to gain knowledge about the course of this disease and understand its pathophysiology, with a focus on Small Fiber neuropathy. Moreover we will validate the german version of the Malmö POTS Score and establish an easy diagnostic scheme for patients in outpatient care.
Inclusion of patients with autoimmune autonomic neuropathies/ pure autonomic failure, postural orthostatic tachycardia syndrome, small fiber neuropathies, Ehlers-Danlos syndromes, Mast cell activation syndrom, Chronic fatigue syndrome and PostCOVID syndrome in our Registry study with follow up visits. Comparison to healthy controls concerning selected examinations.
Planned examinations are laboratory tests, questionnaires on mental and physical health status and circulatory disorders, attention tests, tilt table testing, standing test, sweat function, investigation of small fiber function via quantitative sensory testing as well as the density of nerve fibers in the skin. Measurements are performed at baseline mostly in clinical routine and follow up visits are offered.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Postural Orthostatic Tachycardia Syndrome | Patients with orthostatic intolerance because of Postural orthostatic tachycardia syndrome diagnosed in our outpatient clinic by tilt table examination. |
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| Ehlers Danlos Syndrome | Patients with hypermobile or classical EDS who are already diagnosed including genetical testing for classical or vascular EDS and Marfan Syndromes |
| |
| Autoimmune autonomic neuropathy/Pure autonomic failure | Patients who have an autoimmune autonomic neuropathy based on clinical diagnosis and antibody testing in our outpatient clinic. Cardial MIBG Scintigraphy should have been performed. |
| |
| Healthy controls | Healthy controls with no documented cardiovascular or neurological disorders and no symtoms of autonomic failure/dizziness/fainting | ||
| others | Patients who have comorbidities as mast cell activation syndrome, chronic fatigue and/or Post COVID syndrome, based on clinical diagnosis in our outpatient clinic. |
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| No intervention planned, but all patients get our standart treatment | Other | Patients are selected from clinical Routine and get our standart Treatment based on their disease |
|
| Measure | Description | Time Frame |
|---|---|---|
| Blood pressure | Change in systolic and diastolic blood pressure over time | baseline, 3 months and 18 months |
| Heart frequency | Change in heart frequency over time | baseline, 3 months and 18 months |
| Skin biopsy | intraepithelial nerve fiber density between groups | baseline |
| Composite autonomic severity Score | Change of the Score over time, the results are interpreted as normal (score=0), mild (score=1-3), moderate (score=4-6) or severe (score=7-10) | baseline, 3 months and 18 months |
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Inclusion Criteria:
Exclusion Criteria:
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We will include patients with above mentioned disease who are diagnosed and treated in our outpatient clinic for autonomic disorders in Aachen, Germany.
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Andrea Maier, physician | Contact | +492418089600 | +492418089600 | ans-ambulanz@ukaachen.de |
| Denver Igharo, student | Contact | +492418089600 | +492418089600 | ans-ambulanz@ukaachen.de |
| Name | Affiliation | Role |
|---|---|---|
| Andrea Maier | University Hospital, Aachen | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| University clinic RWTH Aachen | Recruiting | Aachen | North Rhine-Westphalia | 52074 | Germany |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 36437696 | Derived | Igharo D, Thiel JC, Rolke R, Akkaya M, Weis J, Katona I, Schulz JB, Maier A. Skin biopsy reveals generalized small fibre neuropathy in hypermobile Ehlers-Danlos syndromes. Eur J Neurol. 2023 Mar;30(3):719-728. doi: 10.1111/ene.15649. Epub 2022 Dec 13. | |
| 36300662 | Derived | Gerlach DA, Maier A, Manuel J, Bach A, Hoff A, Honemann JN, Heusser K, Voit D, Frahm J, Jordan J, Tank J. Real-Time Magnetic Resonance Imaging to Study Orthostatic Intolerance Mechanisms in Human Beings: Proof of Concept. J Am Heart Assoc. 2022 Nov;11(21):e026437. doi: 10.1161/JAHA.122.026437. Epub 2022 Oct 27. |
| Label | URL |
|---|---|
| official homepage of our outpatient clinic for autonomic nervous system disorders | View source |
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| Paper Skin biopsy reveals generalized small fibre neuropathy in hypermobile Ehlers-Danlos syndromes | View source |
| ID | Term |
|---|---|
| C536196 | Ehlers-Danlos syndrome type 3 |
| D000071075 | Small Fiber Neuropathy |
| D054972 | Postural Orthostatic Tachycardia Syndrome |
| ID | Term |
|---|---|
| D010523 | Peripheral Nervous System Diseases |
| D009468 | Neuromuscular Diseases |
| D009422 | Nervous System Diseases |
| D054971 | Orthostatic Intolerance |
| D054969 | Primary Dysautonomias |
| D001342 | Autonomic Nervous System Diseases |
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