Increasing exercise capacity and physical activity in the COPD patient.

Higher levels of exercise capacity and physical activity are desired outcomes in the comprehensive management of the COPD patient. In addition, improvements in exercise capacity and physical activity are instrumental to optimising other important therapeutic goals, such as improved health status, reduced healthcare utilisation and increased survival. Four general approaches towards increasing exercise capacity and physical activity in individuals with COPD will be discussed in this review: 1) pharmacological intervention, especially the administration of long-acting bronchodilators; 2) pulmonary rehabilitation, including exercise training and collaborative self-management; 3) behavioural interventions; and 4) web-based interventions. These are by no means the only approaches, nor are they mutually exclusive: indeed, combining them, as necessary, to meet the needs of the individual respiratory patient may promote optimal outcomes, although further research is necessary in this area.

Pulmonary Alveolar Proteinosis due to Familial Myelodysplastic Syndrome with resolution after stem cell transplant.

Pulmonary alveolar proteinosis (PAP) is a rare lung disease with an incidence of 0.2 cases per million. PAP has multiple causes, including autoimmune, hereditary, congenital, or secondary. The latter includes hematologic conditions and exposure to different kinds of dust. Most patients present fever, dyspnea, and cough. The chest computed tomography (CT) may reveal the crazy-paving polygonal shapes with superimposed ground glass opacities delimited by thickened interlobular septa; however, this finding is more prevalent in patients with autoimmune PAP. Bronchoalveolar lavage (BAL) shows a milky-opaque appearance with PAS-positive debris on cytology. Treatment is focused on the underlying disease; however, some patients may require whole lung lavage for symptomatic management. We report a case of a 30-year-old female with a history of familial myelodysplastic syndrome (MDS) with GATA 2 mutation who presented to the outpatient clinic with several months of progressive dyspnea and nonproductive cough. The chest CT revealed bilateral ground-glass opacities prominently in the upper lobes. She underwent a bronchoscopy with lavage and biopsy, which revealed fragments of lung parenchyma with intra-alveolar coarse granular eosinophilic material strongly positive for PAS and d-PAS. The overall clinical presentation and histologic findings were diagnostic of PAP. Her GM-CSF was negative, and due to her history of MDS, secondary PAP (S-PAP) was strongly suspected. She underwent a successful allogeneic bone marrow pluripotent stem cell transplant to treat the myelodysplastic syndrome, with a follow-up chest CT showing clear lung parenchyma. The patient had resolution of symptoms about four months after the bone marrow transplant, confirming the diagnosis of S-PAP.

Pulmonary Alveolar Proteinosis due to Familial Myelodysplastic Syndrome with resolution after stem cell transplant

ABSTRACT Pulmonary alveolar proteinosis (PAP) is a rare lung disease with an incidence of 0.2 cases per million. PAP has multiple causes, including autoimmune, hereditary, congenital, or secondary. The latter includes hematologic conditions and exposure to different kinds of dust. Most patients present fever, dyspnea, and cough. The chest computed tomography (CT) may reveal the crazy-paving polygonal shapes with superimposed ground glass opacities delimited by thickened interlobular septa; however, this finding is more prevalent in patients with autoimmune PAP. Bronchoalveolar lavage (BAL) shows a milky-opaque appearance with PAS-positive debris on cytology. Treatment is focused on the underlying disease; however, some patients may require whole lung lavage for symptomatic management. We report a case of a 30-year-old female with a history of familial myelodysplastic syndrome (MDS) with GATA 2 mutation who presented to the outpatient clinic with several months of progressive dyspnea and nonproductive cough. The chest CT revealed bilateral ground-glass opacities prominently in the upper lobes. She underwent a bronchoscopy with lavage and biopsy, which revealed fragments of lung parenchyma with intra-alveolar coarse granular eosinophilic material strongly positive for PAS and d-PAS. The overall clinical presentation and histologic findings were diagnostic of PAP. Her GM-CSF was negative, and due to her history of MDS, secondary PAP (S-PAP) was strongly suspected. She underwent a successful allogeneic bone marrow pluripotent stem cell transplant to treat the myelodysplastic syndrome, with a follow-up chest CT showing clear lung parenchyma. The patient had resolution of symptoms about four months after the bone marrow transplant, confirming the diagnosis of S-PAP.

Mediastinal angiosarcoma presenting as diffuse alveolar hemorrhage.

Angiosarcomas are malignant vascular tumors. Angiosarcomas arising in the thorax such as angiosarcoma of the lungs, heart and mediastinum are extremely rare. There are no reports of mediastinal angiosarcomas presenting with diffuse alveolar hemorrhage, which is a clinical syndrome characterized by the presence of hemoptysis, dyspnea, hypoxia, diffuse lung infiltrates and anemia. Usually, diffuse alveolar hemorrhage is caused by pulmonary capillaritis. Local invasion is more common with these tumors than distant metastasis. These tumors are very aggressive and have a poor response to treatment modalities including surgical resection, chemotherapy, and radiation. Consequently, they carry a poor prognosis. Due to the rarity of these tumors, no standard chemotherapy or radiation protocol exists. We report a case of diffuse alveolar hemorrhage caused by a previously unreported entity: angiosarcoma of the mediastinum.

Climate change and respiratory health.

OBJECTIVE: To discuss the nature of climate change and both its immediate and long-term effects on human respiratory health. METHODS: This review is based on information from a presentation of the American College of Chest Physicians course on Occupational and Environmental Lung Disease held in Toronto, Canada, June 2013. It is supplemented by a PubMed search for climate change, global warming, respiratory tract diseases, and respiratory health. It is also supplemented by a search of Web sites including the Environmental Protection Agency, National Oceanic and Atmospheric Administration, World Meteorological Association, National Snow and Ice Data Center, Carbon Dioxide Information Analysis Center, Inter-Governmental Panel on Climate Change, and the World Health Organization. RESULTS: Health effects of climate change include an increase in the prevalence of certain respiratory diseases, exacerbations of chronic lung disease, premature mortality, allergic responses, and declines in lung function. CONCLUSIONS: Climate change, mediated by greenhouse gases, causes adverse health effects to the most vulnerable patient populations-the elderly, children, and those in distressed socioeconomic strata.

Retinoic acid syndrome.

Vitamin A derivative all-transretinoic acid (ATRA) has been reported to improve the outcome in patients with acute promyelocytic leukemia (APL). Retinoic Acid Syndrome (RAS) is a complication that has been noted to occur during the treatment of APL with ATRA. It is a clinical syndrome consisting of a constellation of signs and symptoms. In a patient with APL on ATRA, the diagnosis of RAS can be made based upon the presence of three of the following features: Fever, dyspnea, weight gain, hypotension, renal failure, pulmonary infiltrates, pleural effusion, and pericardial effusion. Pulmonary manifestations, especially pulmonary edema, are the most common presentation. Incidence of this adverse effect ranges from 6% to 27%. The pathogenesis of this complication is not completely understood. It can be potentially life threatening if not promptly recognized and treated. We report a case of retinoic acid syndrome in a young male with APL being treated with ATRA.