Bases biológicas, patobiológicas y bioclínicas del metabolismo del surfactante pulmonar en la especie humana
Biological, pathobiological and bioclinical bases of pulmonary surfactant metabolism in the human species
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El síndrome de disfunción en el metabolismo del surfactante pulmonar (SMPD), clásicamente denominado Proteinosis Alveolar Pulmonar (PAP), es un desorden respiratorio inusual, caracterizado por una acumulación excesiva y abundante de proteínas y fosfolípidos derivados del surfactante en el alveolo pulmonar y en las vías aéreas distales, con anormalidades asociadas secundarias del intercambio pulmonar gaseoso, que genera insuficiencia respiratoria. Al menos tres mecanismos patofisiológicos generales pueden seguir al desarrollo de los rasgos característicos de la PAP: primario (idiopático, esencial, adquirido o adulto), secundario y congénito.
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