PULMONARY ALVEOLAR PROTEINOSIS IN A HYPOGAMMAGLOBULINEMIC INFANT

Pulmonary alveolar proteinosis (PAP) is a rare disease characterized b y deposition of a surfactant-like material in the alveolar spaces. It is often fatal in infants. PAP has been reported in association with c ellular immunodeficiency syndromes, but not with hypogammaglobulinemia , We present a 10-month-old child with hypogammaglobulinemia in whom P AP developed following cytomegalovirus pneumonitis. Treated with massi ve one-lung lavage, intravenous immunoglobulins, and hydroxychloroquin e, she had prolonged remissions.