RESUMEN
Pulmonary alveolar proteinosis (PAP) is a rare disease with worldwide distribution and an estimated incidence of 0.36 cases per million. We report a case of a PAP coexisting with Pneumocystis jiroveci pneumonia and Mycobacterium tuberculosis infection. The patient was treated with serial lobar lung lavages, GM-CSF, cotrimoxazole, and antituberculosis drugs. His PaO2 on room air improved from 45.7 to 63.8 torr and pulmonary functions normalized (FVC 81.2%, FEV1 95.3%, FEV1/FVC 91.8). A high-resolution computed tomography scan of the thorax showed clearing of both lower lobes. Whole-lung lavage is used in the treatment of PAP, but it may worsen the hypoxemia and lead to hemodynamic instability during the procedure. To the best of our knowledge, there are no reports of bronchoscopic serial lobar lung lavages in cases of PAP performed in India. This method can be performed in bronchoscopic suites having general anesthesia facilities without the requirement of special gadgets.
Asunto(s)
Lavado Broncoalveolar/métodos , Broncoscopía/métodos , Factor Estimulante de Colonias de Granulocitos y Macrófagos/uso terapéutico , Proteinosis Alveolar Pulmonar/terapia , Adulto , Antiinfecciosos/administración & dosificación , Antiinfecciosos/uso terapéutico , Lavado Broncoalveolar/efectos adversos , Factor Estimulante de Colonias de Granulocitos y Macrófagos/administración & dosificación , Humanos , Hipoxia/etiología , India , Masculino , Oxígeno/sangre , Reacción del Ácido Peryódico de Schiff , Neumonía por Pneumocystis/complicaciones , Neumonía por Pneumocystis/diagnóstico , Neumonía por Pneumocystis/tratamiento farmacológico , Proteinosis Alveolar Pulmonar/complicaciones , Proteinosis Alveolar Pulmonar/diagnóstico , Respiración Artificial , Pruebas de Función Respiratoria , Tomografía Computarizada por Rayos X , Combinación Trimetoprim y Sulfametoxazol/administración & dosificación , Combinación Trimetoprim y Sulfametoxazol/uso terapéutico , Tuberculosis Pulmonar/complicaciones , Tuberculosis Pulmonar/diagnóstico , Tuberculosis Pulmonar/tratamiento farmacológicoRESUMEN
The supramitral ring is a rare congenital malformation formed by presence of a ridge of connective tissue, usually attached at or above the mitral annulus. The incidence and clinical presentation is highly variable due to difficulty in diagnosis. A review of autopsied congenital heart diseases at our institute over a 17-year-period revealed 24 cases of supramitral ring. These were classified with respect to the morphology of the ridge and the presence of associated cardiac lesions. The ring was found in 1.5% of the autopsied specimens of congenitally malformed hearts, and in 37.5% of those with obstructed left-sided inflow tracts. The majority of the specimens came from children (79.2%). A clinical diagnosis had been made in only two. In one-third of the cases, the ring was associated with incomplete Shone's complex. Varied anomalies were seen in others, chiefly ventricular septal defects. An interesting association was the presence of rheumatic mitral valvar disease, found in 3 cases. There was no difference in the completeness or width of the supramitral ridge in the hearts from those with or without Shone's complex. Circumferential rings were fleshy and stenosing, while incomplete rings had variable locations and stenosis. The presence of a supramitral ring may be underestimated due to association with other cardiac anomalies, both congenital and acquired. Since the ridge need not always produce stenosis, the correct designation would be simply a supramitral ring.
