Rationale: The pathophysiological changes in respiratory muscle morphology and functioning in patients with end-stage pulmonary disease are not very well known. Furthermore, in COPD, long-term high-intensity NIV is applied without knowing the exact consequences on the lungs and respiratory muscles. Objective: The aims of the study are to get insight in: A. changes respiratory muscles in end-stage respiratory disease, comparing COPD with restrictive lung disease (RLD) due to pulmonary fibrosis B. the effects of long-term HI-NIV in severe COPD patients on the respiratory muscles and the lungs; by comparing COPD patients that had been treated with long-term NIV to COPD patients that were not treated with long-term NIV. Study design: In order to investigate this, the investigators will include in a small pilot cohort study patients being lung transplanted. In these patients there is lung tissue available and respiratory muscle biopsies will be performed during lung-transplant surgery. Study population: Patients that are listed for lung transplantation for an underlying diagnosis of COPD or RLD will be asked to participate. Three groups will be included: patients with a RLD due to pulmonary fibrosis, COPD patients that had been treated with long-term NIV prior to being lung transplanted and COPD patients that were not treated with long-term NIV. Patients will be included definitely once being lung transplanted. Main study parameters/endpoints: The study is an exploratory pilot study. Both contractile strength and the structure of single diaphragm and intercostal muscle fibres as well as lung injury; i.e. alveolar structure and damage and inflammation in the alveoli, will be investigated.
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Respiratory muscle morphology and functioning
Timeframe: during surgery
Ventilator-associated lung injury serum TNF-alfa
Timeframe: during surgery
Ventilator-associated lung injury serum IL-6
Timeframe: during surgery
Ventilator-associated lung injury serum IL-1beta
Timeframe: during surgery
Ventilator-associated lung injury serum IL-8
Timeframe: during surgery
Ventilator-associated lung injury serum MIP-2
Timeframe: during surgery
Ventilator-associated lung injury serum sRAGE
Timeframe: during surgery
Ventilator-associated lung injury BAL TNF-alfa
Timeframe: during surgery
Ventilator-associated lung injury BAL IL-6
Timeframe: during surgery
Ventilator-associated lung injury BAL IL-1beta
Timeframe: during surgery
Ventilator-associated lung injury BAL IL-8
Timeframe: during surgery
Ventilator-associated lung injury BAL MIP-2
Timeframe: during surgery
Ventilator-associated lung injury BAL sRAGE
Timeframe: during surgery
Ventilator-associated lung injury basal membrane
Timeframe: during surgery
Ventilator-associated lung injury alveolar cells
Timeframe: during surgery
Ventilator-associated lung injury edema
Timeframe: during surgery
Ventilator-associated lung injury fibrin
Timeframe: during surgery
Ventilator-associated lung injury inflammatory cells
Timeframe: during surgery
Ventilator-associated lung injury gene-expression
Timeframe: during surgery