Pulmonary alveolar proteinosis: a rare cause of respiratory failure

ABSTRACT

Pulmonary Alveolar Proteinosis [PAP] is a rare syndrome in the paediatric age group and characterized by intra-alveolar accumulation of proteinaceous phospholipid-laden material called surfactant. The diagnosis is made by High Resolution Computed Tomography [HRCT] chest which shows characteristic crazy paving appearance and diagnosis confirmed by Bronchoalveolar Lavage [BAL]. We report two cases. First was a 9-month old infant who presented with respiratory distress and peripheral cyanosis since birth. He was diagnosed on High Resolution Computed Tomography [HRCT] chest as a case of pulmonary alveolar proteinosis and broncho-alveolar lavage confirmed his diagnosis. Second case was a 10-year old female child who had a history of repeated chest infections for 5 years and now presented with cough and respiratory distress for 45 days. She was also diagnosed on HRCT chest but unfortunately she died before bronchoalveolar lavage