Benefits of Pulmonary Rehabilitation in Patients With Severe Lymphangioleiomyomatosis (LAM)

NCT04184193 | Completed

• 1 other identifier: LAM Trial
• Study Type: observational
• Target: 58
• Locations: 1 country
• Sites: 1

Brief Summary

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.

Conditions

Lymphangioleiomyomatosis; LAM; Pulmonary Rehabilitation

Outcome Measures

Primary Outcomes (2)

• 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

Secondary Outcomes (1)

• Change from baseline lung function at week 4

  baseline and week 4

Study Arms (1)

Pulmonary Rehabilitation

Other: Pulmonary Rehabilitation

Interventions

Pulmonary Rehabilitation OTHER

Patients will perform a comprehensive, inpatient pulmonary rehabilitation program for 4 weeks duration

Pulmonary Rehabilitation

Eligibility Criteria

• Age: 18 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)
• Sampling Method: Probability Sample

Study Population

patients with a confirmed diagnosis of Lymphangioleiomyomatosis

You may qualify if:

• confirmed diagnosis of Lymphangioleiomyomatosis
• performed a pulmonary Rehabilitation program at the reference center

You may not qualify if:

• missing data
• repeated stay for pulmonary Rehabilitation at the reference center

Sponsors & Collaborators

• Schön Klinik Berchtesgadener Land: lead

Study Sites (1)

Schoen Klinik Berchtesgadener Land

Schönau am Königssee, Germany

Location

Related Publications (1)

• 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.

  PMID: 32962746 DERIVED

MeSH Terms

Conditions

Lymphangioleiomyomatosis

Condition Hierarchy (Ancestors)

Lymphangiomyoma; Neoplasm, Lymphatic Tissue; Neoplasms by Histologic Type; Neoplasms; Perivascular Epithelioid Cell Neoplasms; Neoplasms, Connective and Soft Tissue; Lymphoproliferative Disorders; Lymphatic Diseases; Hemic and Lymphatic Diseases; Immunoproliferative Disorders; Immune System Diseases

Study Officials

• Rembert A Koczulla, MD

  Schoen Klinik Berchtesgadener Land

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: RETROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Prof. Dr.

Study Record Dates

• First Submitted: November 11, 2019
• First Posted: December 3, 2019
• Study Start: December 3, 2019
• Primary Completion: December 23, 2019
• Study Completion: December 23, 2019
• Last Updated: January 21, 2020

Record last verified: 2020-01

Locations

DE (1)