Lymphangioleiomyomatosis
Conditions
Interventions
Sponsors
Eligibility
Inclusion criteria
Inclusion criteria: All patients followed in the outpatient setting of pulmonary division of Hospital das Clinicas - University of Sao Paulo Medical School, with the following inclusion criteria: 1) Diagnosis of LAM (clinico-radiological or histological) 2) Absence of instability (no exacerbation or hospitalization, for pneumothorax or chylothorax, for example, in the last 6 weeks)
Exclusion criteria
Exclusion criteria: Previous lung transplant Muscular or bone disorder who prevents performing exercise Serious or unstable cardiopathy (functional class IV)
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Evaluation of dynamic hyperinflation during cardiopulmonary exercise testing in patients with lymphangioleiomyomatosis It will be evaluated by periodic measure of inspiratory capacity during the test | — |
Secondary
| Measure | Time frame |
|---|---|
| Evaluation of improve in dynamic hyperinflation during endurance cardiopulmonary exercise testing after administration of inhaled salbutamol. If there is increase in inspiratory capacity during the test Evaluation of increase in exercise tolerance after inhaled salbutamol If there is increase in exercise duration Evaluation of improve in pulmonary funcion tests parameters. Increase in expiratory volume in the first second, reduction in residual volume and in residual volume / total pulmonary capacity ratio. | — |
Countries
Brazil
Contacts
Instituto do Coração do Hospital das Clínicas da Faculdade de Medicina da USP