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Pulmonary Alveolar Proteinosis - Treatment by Serial Whole Lung Lavage - Abstract

Iftikhar A Jan, Nighat Ghias, Nadeem Rizvi, Rhubab Naqvi

 

Abstract

Pulmonary alveolar proteinosis is a rare cause of recurrent chest infections in children. It is characterized by intra alveolar accumulation of lipid and proteinaceous material that is periodic acid Schiff positive. Clinically is associated with respiratory difficulty and derangement of gas exchange. We report the case of a 13 years old boy who presented with recurrent chest infections for the last four years. He was treated in another hospital with antibiotics also for pulmonary tuberculosis due to the respiratory symptoms. On presentation he had dyspnea with signs of respiratory insufficiency. X-ray chest showed bilateral pulmonary involvement with diffuse infiltration of the lung parenchyma. CT scan of lungs showed diffuse alveolar shadowing. Echocardiography showed mild right ventricular hypertrophy. Thoracoscopic biopsy confirmed the diagnosis of pulmonary alveolar proteinosis. The child was treated with four sessions of whole lung saline lavage and showed significant improvements of symptoms. He was well at 6 months follow up.

Keywords: alveolar proteinosis, thoracoscopic biopsy, lung lavage

 

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