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Pulmonary alveolar proteinosis in infants

Y Sakai1, W Abo, H Yoshimura

  • 1Department of Paediatrics, Aomori Central Hospital, Higashi-Tsukurimichi, Japan.

European Journal of Pediatrics
|May 20, 1999
PubMed

Insights

Elevated surfactant protein A in bronchial alveolar lavage fluid may aid in diagnosing infantile pulmonary alveolar proteinosis (PAP) in siblings. This finding suggests a potential diagnostic marker for this rare lung condition.

Area of Science:

  • Pulmonology
  • Genetics
  • Biochemistry

Background:

  • Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by surfactant accumulation.
  • Investigating infantile forms of PAP is crucial for understanding disease mechanisms and diagnosis.

Observation:

  • Two siblings presented with infantile pulmonary alveolar proteinosis (PAP) without congenital respiratory distress.
  • Histological analysis revealed surfactant protein staining in lung tissue.
  • Molecular analysis confirmed messenger RNA expression for surfactant protein B (SP-B), ruling out SP-B defects typical of congenital PAP.

Findings:

  • Elevated concentrations of surfactant protein A (SP-A) were observed in the bronchial alveolar lavage (BAL) fluid of one patient.
  • The absence of SP-B defects suggests a non-congenital form of PAP.

Implications:

  • Elevated SP-A in BAL fluid may serve as a diagnostic clue for infantile PAP.
  • Further research into SP-A's role could refine diagnostic strategies for PAP.
  • Understanding familial PAP cases can illuminate genetic and molecular underpinnings.
Abstract

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