Pulmonary alveolar proteinosis in a child from an informal settlement : 12 litres of fluid drained from the lungs and successful use of ECMO

Abstract

Pulmonary alveolar proteinosis (PAP) is a rare cause of chronic interstitial lung disease, characterised by accumulation of pulmonary surfactant, respiratory insufficiency and an increased incidence of infections. The current standard therapy is whole-lung lavage to remove the accumulated surfactant. We report on a cachexic 12-year-old boy from an informal settlement in South Africa, presenting for the first time with PAP. Twelve litres of broncho-alveolar lavage fluid were drained under extracorporeal membrane oxygenation, and the patient gained 10 kg during his 2-month admission.