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Characteristic aspects of alveolar proteinosis diagnosis Aspectos característicos do diagnóstico da proteinose alveolar

By Thiago Prudente Bártholo, José Gustavo Pugliese, Luiz Carlos Aguiar Vaz, Cláudia Henrique da Costa and Rogério Rufino

Abstract

Alveolar proteinosis is an uncommon pulmonary disease characterized by an accumulation of surfactant in terminal airway and alveoli, thereby impairing gas exchange and engendering respiratory insufficiency in some cases. Three clinically and etiologically distinct forms of pulmonary alveolar proteinosis are recognized: congenital, secondary and idiopathic, the latter corresponding to 90% of the cases. In this case report we present a young male patient that was diagnosed with alveolar proteinosis. Computed tomography of the thorax, bronchoscopy and transbronchial biopsy were performed. The histopathologic aspect was characteristic. The patient was discharged in good health conditions and remains asymptomatic to date.<br>Proteinose alveolar é uma doença pulmonar incomum caracterizada pelo acúmulo de surfactante nas vias aéreas terminais e nos alvéolos, alterando a troca gasosa e, em alguns casos, promovendo insuficiência respiratória. Três formas clínicas e etiologicamente distintas de proteinose alveolar são reconhecidas: congênitas, secundárias e idiopáticas (mais de 90% dos casos são de etiologia idiopática). Neste relato, apresentamos um homem jovem que foi diagnosticado com proteinose pulmonar. Tomografia computadorizada de tórax, broncoscopia e biópsia transbrônquica foram realizadas. O aspecto histopatológico foi característico. O paciente teve alta, com boas condições de saúde, e encontra-se assintomático nos dias de hoje

Topics: Proteinose alveolar, Pavimentação em mosaico, GM-CSF, Pulmonary proteinosis, Crazy paving, GM-CSF, Pathology, RB1-214, Medicine, R, DOAJ:Pathology, DOAJ:Medicine (General), DOAJ:Health Sciences
Publisher: Sociedade Brasileira de Patologia Clínica
Year: 2012
DOI identifier: 10.1590/S1676-24442012000100010
OAI identifier: oai:doaj.org/article:0076bba149ca430c897659653cd9b3d0
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