%0 Journal Article
%T Proteinosis alveolar pulmonar Pulmonary alveolar proteinosis
%A Concepci車n S芍nchez Infante
%A Anixia Serrano S芍nchez
%A Roberto Raz車n Behar
%A Cristina Reyes L車pez
%J Revista Cubana de Pediatrˋ-a
%D 2011
%I Editorial Ciencias M谷dicas
%X La proteinosis alveolar pulmonar es una enfermedad respiratoria cr車nica, caracterizada por alteraci車n en el metabolismo del surfactante, lo que determina su acumulaci車n anormal en el espacio alveolar. Es una enfermedad extremadamente rara. Se han reportado solamente 500 casos en la literatura. Se describi車 por primera vez en 1958. Se presenta un caso de proteinosis alveolar pulmonar en un lactante de 2 meses, con desnutrici車n proteico energ谷tica, que ingresa por dificultad respiratoria e hipoxemia, y, con im芍genes radiol車gicas de tipo ret赤culo-nodulillar, en vidrio deslustrado, en el cual se plantea inicialmente el diagn車stico de bronconeumon赤a. Ante la evoluci車n desfavorable y no respuesta al tratamiento, se realiz車 un estudio para descartar enfermedades pulmonares cr車nicas. El paciente fallece y se confirma el diagn車stico por anatom赤a patol車gica. Se realiza una revisi車n del tema. The pulmonary alveolar proteinosis is a chronic respiratory disease characterized by surfactant metabolism alteration determining its abnormal accumulation in the alveolar space. It is a disease very rare and in literature only 500 cases have been reported; it was described for the first time in 1958. This is a case presentation of pulmonary alveolar proteinosis in an infant aged 2 months with energetic protein malnutrition admitted due to respiratory difficulty and hypoxemia and with radiologic images of the reticulonodulillary, in frosting glass, where initially is made the diagnosis of bronchopneumonia. In the face of unfavorable evolution and no response to treatment, a study was conducted to rule out chronic pulmonary diseases. Patient died confirming the diagnosis according to the pathologic anatomy. A review on subject is carried out.
%K proteinosis alveolar pulmonar
%K PAP
%K bronconeumon赤a
%K pulmonary alveolar proteinosis
%K PAP
%K bronchopneumonia
%U http://scielo.sld.cu/scielo.php?script=sci_arttext&pid=S0034-75312011000400009