This preliminary report suggests that the use of Bosentan in the treatment of PH in COPD is beneficial.

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Background: Weight loss and skeletal muscle wasting are common in patients with chronic obstructive pulmonary disease (COPD) and can influence the course and the prognosis of COPD. Hypophosphatemia is a pathologic status often characterized by muscle weakness and is a frequent laboratory finding in these patients. Objective: The aim of the present study was to evaluate the effect of an organic phosphate (fructose 1,6-diphosphate, FDP) administration on respiratory performance in 45 malnourished COPD patients in stable clinical conditions. Methods: Physiologic evaluation including spirometry, maximal voluntary ventilation (MMV), elevated arm test, maximal mouth pressures (PImax and PEmax), respiratory response to CO₂, oxygen (PaO₂) and carbon dioxide (PaCO₂) arterial tension, a visual analogic scale (VAS) to measure dyspnea, and complete blood tests were done at the beginning and again at the end of the study. Results: After FDP administration, there was a significant increase in PImax (43.0 ± 18.3 cm H₂O before treatment vs. 49.8 ± 14.9 cm H₂O after treatment; p < 0.005). This did not occur in the placebo group (40.3 ± 17.4 cm H₂O before treatment vs. 42.6 ± 20.1 cm H₂O after treatment, nonsignificant). There was also a trend of VAS to decrease and of MVV to increase. Conclusions: These results show that FDP administration may be useful in the management of malnourished COPD patients, especially in increasing their respiratory muscle strength.

Backgrounds: In Chronic Obstructive Pulmonary Disease (COPD) a multi factorial effort limitation becomes progressively relevant as the disease progresses in the consecutive stages. It is measured by both six minutes walking test (6MWT) and maximal cardiopulmonary incremental test (CPET). Aim: It is important to assess in each stage of disease the metabolic load during 6MWT referring to the outcome of CPET and to ascertain whether there is a significant relationship between the measures obtained by CPET and 6MWT. Methods: Four groups of fifteen patients affected by COPD in stage I to IV underwent 6MWT and maximal CPET in the same day and results were compared to a group of healthy people. Airflow obstruction was measured by whole body plethysmography, blood gases by gas analysis, maximal oxygen consumption and metabolic parameters by ergometer, lactic acid levels by analyzer. Results: Maximal oxygen consumption (V'O 2 max) and 6MWT are progressively impaired and related (V'O 2 max = 1.25  0.26, 1.152  0.4, 1.03  0.44, .85  0.2 l/m; 6MWD = 452  84, 446  82, 381  165, 200  100 respectively in GOLD I to IV stage). Oxygen consumption (V'O 2) during 6MWT becomes stable after 3-4 minutes and reached at the end of the test close to those measured at decompensated metabolic acidosis anaerobic threshold (TDMA) (85 ± 0.4 l/m vs. 9  0.4 l/m) in stage I to III, while in COPD there is no difference between V'O 2 max and V'O 2 during 6MWT (0.85  0.2 vs. 0.8  0.23 l/m). 6MWT is more suitable to determine oxygen desaturation than CPET (dSaO 2 −4 ± 2% vs. −2 ± 1%). 6MWD, the workload performed in 6MWT and V'O 2 max are significantly related. Conclusion: 6MWT looks as a suitable sub maximal test related CPET. Metabolic requirements under 6MWT are close to TDMA and are obtained in a suitable, self paced, usual exercise, close to everyday experience and thus related to activity daily levels. As the disease worsens the differences between V'O 2 during 6MWT and V'O 2 max wane.