Abstract
Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by deposition of a surfactant-like material in the alveolar spaces. It is often fatal in infants. PAP has been reported in association with cellular immunodeficiency syndromes, but not with hypogammaglobulinemia. We present a 10-month-old child with hypogammaglobulinemia in whom PAP developed following cytomegalovirus pneumonitis. Treated with massive one-lung lavage, intravenous immunoglobulins, and hydroxychloroquine, she had prolonged remissions.
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