Congenital surfactant protein B deficiency--emphasis on imaging.
OBJECTIVE
Surfactant protein B deficiency has become increasingly recognized as a cause of severe prolonged respiratory distress. Little has been written about the imaging appearance and the role of imaging in diagnosis.


METHODS
Three newborn infants with severe respiratory distress exhibited prolonged diffuse pulmonary opacification radiographically.


RESULTS
Diffuse ground-glass opacity and markedly prominent interlobular septa suggesting alveolar proteinosis were present on early thin-section chest computed tomographic (CT) images. Fibrotic changes with prominent interlobular septal thickening were present on a later CT. Surfactant protein B deficiency was confirmed by alveolar lavage and peripheral blood DNA analysis.


CONCLUSIONS
Thin-section chest CT imaging contributes important information when this diagnosis is considered.
Newman
B
B
Department of Radiology, Children's Hospital of Pittsburgh, 3705 Fifth Avenue, Pittsburgh, PA 15213, USA. Newmanb@chplink.chp.edu
Kuhn
J P
JP
Kramer
S S
SS
Carcillo
J A
JA
D015199:Extracorporeal Membrane Oxygenation; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009154:Mutation; D011498:Protein Precursors / Q000172:deficiency* / Q000235:genetics / Q000378:metabolism; D011510:Proteolipids / Q000235:genetics / Q000378:metabolism; D011649:Pulmonary Alveolar Proteinosis / Q000000981:diagnostic imaging* / Q000209:etiology* / Q000235:genetics; D014057:Tomography, X-Ray Computed
D011498:Protein Precursors; D011510:Proteolipids; C105511:surfactant protein B propeptide