Pulmonary alveolar proteinosis in children.
Pulmonary alveolar proteinosis (PAP) is a rare cause of chronic interstitial lung disease in children characterised by accumulation of a lipoproteinaceous material in the alveoli. Bronchoalveolar lavage is the key diagnostic tool, revealing a milky appearance of the return fluid and a periodic acid-Schiff staining material in the alveolar macrophages. PAP is a heterogeneous disease. Immediate-onset forms leading to early and fatal respiratory failure may be related to SP-B deficiency. Postnatal-onset PAP may be associated with various diseases or may be primary. The latter has a polymorphic progression from asymptomatic to uncontrollable respiratory failure. Recent studies have implicated GM-CSF and/or its receptor but the exact underlying mechanisms are still unknown. Therapeutic lung lavages are the only effective treatment for severe cases.
de Blic
Jacques
J
Service de Pneumologie et Allergologie Pédiatriques, Hôpital Necker Enfants Malades, 149 rue de Sèvres 75015, Paris, France. j.deblic@nck.ap-hop-paris.fr
D017668:Age of Onset; D018893:Bronchoalveolar Lavage; D018450:Disease Progression; D005260:Female; D006801:Humans; D007223:Infant; D010503:Periodic Acid-Schiff Reaction; D011649:Pulmonary Alveolar Proteinosis / Q000175:diagnosis* / Q000453:epidemiology / Q000209:etiology / Q000628:therapy