Pulmonary alveolar proteinosis in infants.
We investigated the histological and molecular characteristics of pulmonary alveolar proteinosis (PAP) in two siblings (a brother and sister) who did not exhibit respiratory distress at birth but who each developed symptoms during infancy. Histological analysis of lung specimens showed positive staining for surfactant proteins in both patients. The polymerase chain reaction revealed expression of messenger RNA for surfactant protein B (SP-B) in the lung specimens. No defect in SP-B which is characteristic of the congenital form of PAP was observed. The concentration of surfactant protein A (SP-A) in bronchial alveolar lavage (BAL) fluid was elevated in patient 1 suggesting the BAL concentration of SP-A may be a clue to the diagnosis of this form of PAP.


CONCLUSIONS
The accumulation of surfactant protein A in two siblings with an infantile form of pulmonary alveolar proteinosis could be a clue to the diagnosis.
Sakai
Y
Y
Department of Paediatrics, Aomori Central Hospital, Higashi-Tsukurimichi, Japan.
Abo
W
W
Yoshimura
H
H
Sano
H
H
Kuroki
Y
Y
Satoh
M
M
Kaimori
M
M
D001706:Biopsy; D001992:Bronchoalveolar Lavage Fluid / Q000737:chemistry; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D007223:Infant; D008297:Male; D016133:Polymerase Chain Reaction; D011510:Proteolipids / Q000378:metabolism*; D011649:Pulmonary Alveolar Proteinosis / Q000378:metabolism* / Q000473:pathology*; D037662:Pulmonary Surfactant-Associated Protein A; D037661:Pulmonary Surfactant-Associated Proteins; D011663:Pulmonary Surfactants / Q000378:metabolism*; D012313:RNA / Q000032:analysis
D011510:Proteolipids; D037662:Pulmonary Surfactant-Associated Protein A; D037661:Pulmonary Surfactant-Associated Proteins; D011663:Pulmonary Surfactants; D012313:RNA