RESUMEN
Sandhoff disease, Gaucher disease type I and sialidosis type I are lysosomal storage disorders caused, respectively, by deficiency of activity of beta-hexosaminidase (storage of GM(2) and GA(2) ganglioside), glucosylceramidase (storage of glucosylceramide) and alpha-neuraminidase (storage of glucopeptides and/or oligosaccharides). Progressive clinical systemic and neurological dysfunctions are observed. In these pathologies, respiratory infections often lead to death. Elevation of the lung surfactant phosphatidylcholine (PC) has previously been reported in the Hexb mouse, a model of Sandhoff disease. We evaluated phospholipids in the lung surfactant of patients affected by the described lysosomal diseases, observing a statistically significant increase of total lipid phosphate in the patients as compared with controls. Moreover, higher levels of PC in patients affected by sialidosis (3.6-fold) and Gaucher (4-fold) disease, and of PC (4.15-fold) and phosphatidylethanolamine (2.3-fold) in a patient affected by Sandhoff disease were noted. The latter confirms the previous results in the Hexb mouse. We suggest that changes in phospholipid metabolism can be common in different lysosomal storage disorders and can increase the susceptibility to respiratory infections, usually present in these disorders.
Asunto(s)
Pulmón/patología , Enfermedades por Almacenamiento Lisosomal/metabolismo , Fosfatidilcolinas/metabolismo , Surfactantes Pulmonares/metabolismo , Animales , Enfermedad de Gaucher/metabolismo , Humanos , Lípidos/química , Pulmón/metabolismo , Ratones , Fosfatidiletanolaminas/metabolismo , Fosfolípidos/metabolismoAsunto(s)
Mesotelioma/patología , Neoplasias Pleurales/patología , Adulto , Humanos , Inmunohistoquímica , Masculino , Mesotelioma/diagnóstico por imagen , Mesotelioma/metabolismo , Microscopía Electrónica , Neoplasias Pleurales/diagnóstico por imagen , Neoplasias Pleurales/metabolismo , Radiografía , Vimentina/metabolismoRESUMEN
Doze pacientes asmáticas com dispnéia noturna e/ou matutina foram tratados com fenoterol (400mcg 3x dia) ou salbutamol (200 mcg 3 x dia), ambos sob a forma de aerosol dosificador, três dias para cada droga, com um intervalo de 24 horas de "wash-out" entre uma droga e outra. A análise dos dados ventilatórios FEV1 - FEF 25-75 - FEF) e do sintoma dispnéia evidenciou constantemente uma maior intensidade e duraçäo de açäo broncoespasmolítica do fenoterol em relaçäo ao salbutamol
Asunto(s)
Adulto , Humanos , Masculino , Femenino , Albuterol/administración & dosificación , Espasmo Bronquial/tratamiento farmacológico , Fenoterol/administración & dosificación , Aerosoles , Ensayos Clínicos como AsuntoRESUMEN
Today it is believed that mast cells (MC) are important not only in IgE-mediated reactions, but also in delayed hypersensitivity reactions, and that their functions are mediated by factors released by T lymphocytes. Recent studies have shown their presence in bronchoalveolar lavage (BAL) of patients with asthma and interstitial lung disease. MC have been identified by us in the BAL of patients with sarcoidosis and lung cancer, and in controls. A statistically significant correlation has been found between MC and lymphocytes, CD3+ and CD4+ cells present in BAL, thus supporting the hypothesis of interactions between T lymphocytes and MC in immune reactions at the alveolar level.