Article
Absence of lamellar bodies with accumulation of dense bodies characterizes a novel form of congenital surfactant defect.
Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society - 1 Jan 2000
Tryka A F, Wert S E, Mazursky J E, Arrington R W, Nogee L M
Abstract excerpt
Two female sibling full-term newborns developed respiratory distress shortly after birth, which progressed to respiratory failure. Tracheal lavage demonstrated presence of surfactant protein A (SP-A), but little surfactant protein B (SP-B), without aberrant surfactant protein C (SP-C). On a lung biopsy performed in both infants, prominent type II pneumocyte hyperplasia was evident. Through ultrastructural...
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