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Data from patients with the orphan disease of lymphangioleiomyomatosis (LAM) which performed a pulmonary Rehabilitation program will be analyzed retrospectively. Data will be taken from the internal data base of the reference Center (Schoen Klinik Berchtesgadener Land, Schoenau, Germany) where These data were collected during clinical routine. Data will be included from the year 2000 until now. A retrospectively matched COPD cohort will be included for comparison.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Pulmonary Rehabilitation |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Pulmonary Rehabilitation | Other | Patients will perform a comprehensive, inpatient pulmonary rehabilitation program for 4 weeks duration |
|
| Measure | Description | Time Frame |
|---|---|---|
| Change from baseline 6 Minute walk distance at week 4 | the walked distance in meter will be the Primary outcome of the 6 Minute walk test | baseline and week 4 |
| Change from baseline Quality of life at week 4 | the physical and mental health component summary score from the Short Form 36 Health Questionnaire will be recorded. Score range from 0 to 100 with lower scores indicating worse impairment | baseline and week 4 |
| Measure | Description | Time Frame |
|---|---|---|
| Change from baseline lung function at week 4 | forced expiratory volume in 1 second and inspiratory vital capacity will be provided in Liter and percent predicted | baseline and week 4 |
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Inclusion Criteria:
Exclusion Criteria:
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patients with a confirmed diagnosis of Lymphangioleiomyomatosis
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| Name | Affiliation | Role |
|---|---|---|
| Rembert A Koczulla, MD | Schoen Klinik Berchtesgadener Land | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Schoen Klinik Berchtesgadener Land | Schönau am Königssee | Germany |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 32962746 | Derived | Gloeckl R, Nell C, Schneeberger T, Jarosch I, Boensch M, Watz H, Wirtz H, Welte T, Kenn K, Koczulla AR. Benefits of pulmonary rehabilitation in patients with advanced lymphangioleiomyomatosis (LAM) compared with COPD - a retrospective analysis. Orphanet J Rare Dis. 2020 Sep 22;15(1):255. doi: 10.1186/s13023-020-01540-3. |
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| ID | Term |
|---|---|
| D018192 | Lymphangioleiomyomatosis |
| ID | Term |
|---|---|
| D008203 | Lymphangiomyoma |
| D018190 | Neoplasm, Lymphatic Tissue |
| D009370 | Neoplasms by Histologic Type |
| D009369 | Neoplasms |
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| D054973 |
| Perivascular Epithelioid Cell Neoplasms |
| D018204 | Neoplasms, Connective and Soft Tissue |
| D008232 | Lymphoproliferative Disorders |
| D008206 | Lymphatic Diseases |
| D006425 | Hemic and Lymphatic Diseases |
| D007160 | Immunoproliferative Disorders |
| D007154 | Immune System Diseases |