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Paediatr Respir Rev. 2004 Dec;5(4):316-22.

Pulmonary alveolar proteinosis in children.

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Service de Pneumologie et Allergologie Pédiatriques, Hôpital Necker Enfants Malades, 149 rue de Sèvres 75015, Paris, France.


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.

[Indexed for MEDLINE]

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