Prospective study to evaluate quality of life in amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative rare disease characterized by symptoms and signs in the upper and lower motor neurons, leading to progressive neuro-degeneration and muscle atrophy. Our objective was to analyse the quality of life (QoL) in patients with ALS and compare with general population and with patients with cancer. Prospective study from consecutive ALS patients in one center.

Practical Recommendations for Diagnosis and Management of Respiratory Muscle Weakness in Late-Onset Pompe Disease.

Pompe disease is an autosomal-recessive lysosomal storage disorder characterized by progressive myopathy with proximal muscle weakness, respiratory muscle dysfunction, and cardiomyopathy (in infants only). In patients with juvenile or adult disease onset, respiratory muscle weakness may decline more rapidly than overall neurological disability. Sleep-disordered breathing, daytime hypercapnia, and the need for nocturnal ventilation eventually evolve in most patients.

Randomized trial of non-invasive ventilation combined with exercise training in patients with chronic hypercapnic failure due to chronic obstructive pulmonary disease.

Background: Non-invasive ventilation and exercise training might prove beneficial in the management of COPD patients.

Objetive: to compare the combined use of exercise training and non-invasive ventilation with the two interventions separately in chronic respiratory failure due to chronic obstructive pulmonary disease. As primary objective exercise capacity and secondary objectives gas exchange, peripheral muscle strength, BODE index, quality of life and systemic inflammatory response.

Rivaroxaban for the treatment of venous thromboembolism. A "real-life" perspective in 103 patients.

Introduction: Randomized clinical trials have demonstrated non-inferiority of rivaroxaban compared with vitamin K antagonists (VKAs) in the treatment of venous thromboembolism (VTE). Our objective was to analyze in real life, tolerance, recurrence, bleeding and adverse events of rivaroxaban in patients with acute symptomatic VTE.

Material And Methods: Open follow-up study of a cohort of patients aged 18 and over diagnosed with deep vein thrombosis (DVT) and/or pulmonary embolism (PE) treated with rivaroxaban from December 2011 to January 2014.

[Outpatient management of patients with deep vein thrombosis and cancer: a study of safety, cost and budget impact].

Background And Objective: This is a safety and cost comparison study with an analysis of budgetary impact of ambulatory management of patients with cancer and deep vein thrombosis (DVT) compared with hospital management.

Material And Methods: Prospective observational study of patients with known malignancy and diagnosed with DVT from 2003 to 2007. The outcome variables were mortality, relapse and bleeding in one month.

Components of physical capacity in patients with chronic obstructive pulmonary disease: relationship with phenotypic expression.

Background: More accurate phenotyping of COPD is of great interest since it may have prognostic and therapeutic consequences. We attempted to explore the possible relationship between the extent of emphysema, as assessed by high-resolution computed tomography (HRCT), and COPD severity. We also included some study variables involving exercise tolerance evaluation and peripheral muscle strength (PMS) measurement.

Overexpression of Aquaporin-1 in lung adenocarcinomas and pleural mesotheliomas.

Aquaporin-1 (AQP1) is the main water channel responsible for water transport through many epithelia and endothelia. The latest evidence pointed toward an important role of this protein also in gas permeation, angiogenesis, cell proliferation and migration. In the present work we studied the expression of AQP1 by immunohistochemical staining of 92 lung biopsies from patients diagnosed with a pleuro-pulmonary tumor (71 lung and 21 pleural neoplasms).

Clinical consequences of reduced dosing schedule during treatment of a patient with Pompe's disease.

Introduction: Pompe's disease is a metabolic myopathy caused by a deficiency of the enzyme alpha-glucosidase. Patients with late-onset Pompe's disease have progressive muscle weakness, which also affects pulmonary function. Since the advent of specific treatment for Pompe's disease, enzyme replacement therapy with alpha-glucosidase, the prognosis of the disease has changed.

Testing for occult cancer in patients with pulmonary embolism: results from a screening program and a two-year follow-up survey.

Introduction: An association between pulmonary embolism (PE) and a subsequent diagnosis of cancer has been repeatedly reported. Although screening and early detection might play a pivotal part in reducing mortality from cancer, there are currently no definite data to suggest that cancer screening may improve survival rates in patients with PE. We hereby present the results of a screening program and a two-year follow-up survey for detecting occult cancer in this patient population.

[Health-related quality of life of patients receiving home mechanical ventilation: the Spanish version of the severe respiratory insufficiency questionnaire].

Objective: Home mechanical ventilation is used with patients with severe, chronic respiratory failure, a condition that has a serious impact on quality of life. The aim of this study was to produce a translation and cultural adaptation of the Severe Respiratory Insufficiency. Questionnaire for the Spanish population, the first health-related quality of life questionnaire specifically designed for patients receiving home mechanical ventilation.