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Primary alveolar capillary dysplasia (acinar dysplasia) and surfactant protein B deficiency: a clinical, radiological and pathological study

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Abstract

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.

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Author notes

  1. Claes O. Hugosson

    Present address: , Jaktstigen 5, 18146, Lidingö, Sweden

Authors and Affiliations

  1. Department of Radiology MBC#28, King Faisal Specialist Hospital and Research Centre, 3354, 11211, Riyadh, Saudi Arabia

    Claes O. Hugosson & Nuha Khoumais

  2. Department of Paediatrics, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia

    Husam M. Salama & Abdul H. Kattan

  3. Department of Pathology, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia

    Fouad Al-Dayel

Authors
  1. Claes O. Hugosson
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  2. Husam M. Salama
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  3. Fouad Al-Dayel
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  4. Nuha Khoumais
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  5. Abdul H. Kattan
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Correspondence to Claes O. Hugosson.

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Hugosson, C.O., Salama, H.M., Al-Dayel, F. et al. Primary alveolar capillary dysplasia (acinar dysplasia) and surfactant protein B deficiency: a clinical, radiological and pathological study. Pediatr Radiol 35, 311–316 (2005). https://doi.org/10.1007/s00247-004-1349-7

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  • DOI: https://doi.org/10.1007/s00247-004-1349-7

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