BACKGROUND: Full-term infants with severe and prolonged respiratory distress represent a diagnostic challenge. Plain radiographic findings may be nonspecific or similar to classic surfactant deficiency disease for infants with surfactant protein B deficiency and acinar dysplasia. OBJECTIVES: To describe the similar clinical-radiolgical patterns of two rare neonatal conditions. MATERIALS AND METHODS: Six newborn babies with severe respiratory distress at birth demonstrated clinical and radiographically prolonged and progressive diffuse pulmonary opacification. RESULTS: All infants demonstrated hyperinflation of the lungs. The diffuse hazy opacification, which varied from mild (n=3) to moderate (n=3), progressed to severe diffuse opacification preceding death, which occurred at 12-36 days of life. Open lung biopsy confirmed the diagnosis of primary alveolar acinar dysplasia (AD) in four infants and surfactant protein B deficiency (SPBD) in two infants. CONCLUSIONS: In full-term babies with unexplained progressive respiratory distress from birth and progress of radiological changes, both AD and SPBD should be considered.
BACKGROUND: Full-term infants with severe and prolonged respiratory distress represent a diagnostic challenge. Plain radiographic findings may be nonspecific or similar to classic surfactant deficiency disease for infants with surfactant protein B deficiency and acinar dysplasia. OBJECTIVES: To describe the similar clinical-radiolgical patterns of two rare neonatal conditions. MATERIALS AND METHODS: Six newborn babies with severe respiratory distress at birth demonstrated clinical and radiographically prolonged and progressive diffuse pulmonary opacification. RESULTS: All infants demonstrated hyperinflation of the lungs. The diffuse hazy opacification, which varied from mild (n=3) to moderate (n=3), progressed to severe diffuse opacification preceding death, which occurred at 12-36 days of life. Open lung biopsy confirmed the diagnosis of primary alveolar acinar dysplasia (AD) in four infants and surfactant protein B deficiency (SPBD) in two infants. CONCLUSIONS: In full-term babies with unexplained progressive respiratory distress from birth and progress of radiological changes, both AD and SPBD should be considered.
Authors: Mohammed Tredano; Matthias Griese; Jacques de Blic; Tifenn Lorant; Claude Houdayer; Silja Schumacher; François Cartault; Frédérique Capron; Liliane Boccon-Gibod; Thierry Lacaze-Masmonteil; Sylvain Renolleau; Bertrand Delaisi; Jacques Elion; Rémy Couderc; Michel Bahuau Journal: Am J Med Genet A Date: 2003-06-15 Impact factor: 2.802
Authors: D E deMello; L M Nogee; S Heyman; H F Krous; M Hussain; T A Merritt; W Hsueh; J E Haas; K Heidelberger; R Schumacher Journal: J Pediatr Date: 1994-07 Impact factor: 4.406
Authors: Jason P Weinman; Christina J White; Deborah R Liptzin; Robin R Deterding; Csaba Galambos; Lorna P Browne Journal: Pediatr Radiol Date: 2018-04-23
Authors: Gabriel Nuncio Benevides; Patricia Picciarelli de Lima; Aloisio Felipe-Silva; Silvana Maria Lovisolo; Ana Maria Andrello Gonçalves Pereira de Melo Journal: Autops Case Rep Date: 2015-03-30