ABSTRACT
Pulmonary alveolar proteinosis is a progressive disease recognized 35 years ago. Many etiologies have been put forward, but the pathogenesis remains obscure. Familial alveolar proteinosis is a rare condition, probably transmitted by recessive autosomal inheritance, with deficiency in surfactant apoprotein SPB or defective expression of the common granulocyte colony stimulating factor (GM-CSF), IL3 and IL5 receptor. Gene therapy could be a future option for treatment in patients with specific gene defects.
Subject(s)
Pulmonary Alveolar Proteinosis/diagnosis , Adult , Bronchoalveolar Lavage , Female , Humans , Immunoglobulin G/analysis , Pulmonary Alveolar Proteinosis/genetics , Remission, SpontaneousABSTRACT
Askin tumor is uncommon, usually observed in young subjects. We report two cases of Askin tumor observed in patients aged 15 and 27 years who were hospitalized for a painful tumefaction of the chest wall with pleural involvement in one patient. We reviewed progress in patient management which has resulted from better understanding of the natural history of Askin tumor and earlier diagnosis due to advances in imaging and immunohistochemistry techniques as well as cytogenetics. A number of points remain controversial.
Subject(s)
Carcinoma, Small Cell/diagnosis , Neuroectodermal Tumors, Primitive, Peripheral/diagnosis , Thoracic Neoplasms/diagnosis , Adolescent , Adult , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Biopsy, Needle , Carcinoma, Small Cell/complications , Carcinoma, Small Cell/therapy , Chest Pain/etiology , Cytogenetics , Fatal Outcome , Female , Hemoptysis/etiology , Humans , Immunohistochemistry , Neuroectodermal Tumors, Primitive, Peripheral/complications , Neuroectodermal Tumors, Primitive, Peripheral/therapy , Radiotherapy, Adjuvant , Thoracic Neoplasms/complications , Thoracic Neoplasms/therapy , Tomography, X-Ray ComputedABSTRACT
Pleuropulmonary amibiasis is generally secondary to hepatic amibiasis with migration into the thorax. Direct pulmonary involvement is exceptional. We report a case of pulmonary ambiasis without associated liver involvement causing multiple bilateral pulmonary lesions.
Subject(s)
Amebiasis/diagnostic imaging , Lung Diseases, Parasitic/diagnostic imaging , Adult , Female , Humans , RadiographyABSTRACT
Five case histories of intra-thoracic involvement occurring in the course of Behcet's disease were studied. These included a superior vena caval obstruction, an obstructive syndrome, transitory infiltrates, repeated lung infections and a case of haemoptysis. A review of the literature was carried out "à propos" of each case. The blood results seemed to be the involved as a manifestation of all the varied clinical presentations. The advantage of specialised investigations such as pulmonary angiography, perfusion scintigraphy and bronchial arteriography is underlined.
Subject(s)
Behcet Syndrome/complications , Lung Diseases/etiology , Adult , Airway Obstruction/etiology , Female , Hemoptysis/etiology , Humans , Lung Diseases/diagnostic imaging , Male , Middle Aged , Pleural Effusion/etiology , Radiography , Superior Vena Cava Syndrome/diagnostic imaging , Superior Vena Cava Syndrome/etiologyABSTRACT
The pH, the PCO2 and the PO2 of pleural liquid in 100 patients in the pneumology unit of the Sfax Hospital been measured. Variations according to etiology have been noticed: 44 tubercular pleurisies have presented an acid pH, a high PCO2 and a low PO2, without variation according of the evolution; 34 cancerous pleurisies were divided as follow: recent pleurisies with alcaline pH, normal PO2 and PCO2; neoplastic pleurisies with developed degree with acid pH, low PO2 and high PCO2; 6 cases of cardiac pleurisies have presented a very high PO2; some other etiologies have been explored (bacterial, viral, cirrhotic, rheumatoid, pancreatic). Afterwards, we have tried to bring out some physio-pathological explanations. At the end of this study, the gazometry of pleural liquid, of easy execution and rapid result, seems to be a useful complementary biological test.
