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Surfactant Deficiency Disease
Hank Baskin, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Surfactant deficiency disease (SDD)
            • Synonyms

              • Lung disease of prematurity
              • Respiratory distress syndrome
              • Hyaline membrane disease (archaic)
            • Definitions

              • Prematurity-related SDD (most common usage)
                • Surfactant deficiency in premature newborns
                • Lungs begin producing surfactant at ~ 24-weeks gestational age (GA); adequate amounts are usually present by 36-weeks GA
                • Lack of sufficient surfactant causes diffuse microatelectasis & abnormal pulmonary compliance
              • Secondary SDD (less common usage)
                • Meconium aspiration or pneumonia can cause surfactant deactivation
                • Results are similar to those seen in prematurity, but usually heterogeneous & superimposed on other findings
              • Primary SDD (least common usage)
                • Abnormal gene products adversely affect composition &/or function of surfactant
                • Often termed surfactant dysfunction disorders
                • Most well-known causes: Surfactant protein-B, surfactant protein-C, ABCA3, & TTF-1 deficiencies
                • Synergistically ↓ outcome with prematurity & 1° SDD

            IMAGING

            • General Features

              • Radiographic Findings

                • Ultrasonographic Findings

                  • CT Findings

                    • Other Modality Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            CLINICAL ISSUES

                            • Presentation

                              • Demographics

                                • Natural History & Prognosis

                                  • Treatment

                                    Selected References

                                    1. Ma H et al: Diagnostic value of lung ultrasound for neonatal respiratory distress syndrome: a meta-analysis and systematic review. Med Ultrason. 22(3):325-33, 2020
                                    2. Magnani JE et al: Persistent respiratory distress in the term neonate: genetic surfactant deficiency diseases. Curr Pediatr Rev. 16(1):17-25, 2020
                                    3. Wu J et al: Lung ultrasound for the diagnosis of neonatal respiratory distress syndrome: a meta-analysis. Ultrasound Q. 36(2):102-10, 2020
                                    4. Corsini I et al: Lung ultrasound for the differential diagnosis of respiratory distress in neonates. Neonatology. 115(1):77-84, 2019
                                    5. Grimaldi C et al: Thoracic ultrasound accuracy for the investigation of initial neonatal respiratory distress. Arch Pediatr. 26(8):459-65, 2019
                                    6. Somaschini M et al: Surfactant proteins gene variants in premature newborn infants with severe respiratory distress syndrome. J Perinatol. 38(4):337-44, 2018
                                    7. Chen SW et al: Routine application of lung ultrasonography in the neonatal intensive care unit. Medicine (Baltimore). 96(2):e5826, 2017
                                    8. Hiles M et al: Neonatal respiratory distress syndrome: chest X-ray or lung ultrasound? A systematic review. Ultrasound. 25(2):80-91, 2017
                                    9. Kröner C et al: Lung disease caused by ABCA3 mutations. Thorax. 72(3):213-20, 2017
                                    10. Sardesai S et al: Evolution of surfactant therapy for respiratory distress syndrome: past, present, and future. Pediatr Res. 81(1-2):240-8, 2017
                                    Related Anatomy
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                                    Related Differential Diagnoses
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                                    References
                                    Tables

                                    Tables

                                    KEY FACTS

                                    • Terminology

                                      • Imaging

                                        • Top Differential Diagnoses

                                          • Pathology

                                            • Clinical Issues

                                              TERMINOLOGY

                                              • Abbreviations

                                                • Surfactant deficiency disease (SDD)
                                              • Synonyms

                                                • Lung disease of prematurity
                                                • Respiratory distress syndrome
                                                • Hyaline membrane disease (archaic)
                                              • Definitions

                                                • Prematurity-related SDD (most common usage)
                                                  • Surfactant deficiency in premature newborns
                                                  • Lungs begin producing surfactant at ~ 24-weeks gestational age (GA); adequate amounts are usually present by 36-weeks GA
                                                  • Lack of sufficient surfactant causes diffuse microatelectasis & abnormal pulmonary compliance
                                                • Secondary SDD (less common usage)
                                                  • Meconium aspiration or pneumonia can cause surfactant deactivation
                                                  • Results are similar to those seen in prematurity, but usually heterogeneous & superimposed on other findings
                                                • Primary SDD (least common usage)
                                                  • Abnormal gene products adversely affect composition &/or function of surfactant
                                                  • Often termed surfactant dysfunction disorders
                                                  • Most well-known causes: Surfactant protein-B, surfactant protein-C, ABCA3, & TTF-1 deficiencies
                                                  • Synergistically ↓ outcome with prematurity & 1° SDD

                                              IMAGING

                                              • General Features

                                                • Radiographic Findings

                                                  • Ultrasonographic Findings

                                                    • CT Findings

                                                      • Other Modality Findings

                                                        • Imaging Recommendations

                                                          DIFFERENTIAL DIAGNOSIS

                                                            PATHOLOGY

                                                            • General Features

                                                              CLINICAL ISSUES

                                                              • Presentation

                                                                • Demographics

                                                                  • Natural History & Prognosis

                                                                    • Treatment

                                                                      Selected References

                                                                      1. Ma H et al: Diagnostic value of lung ultrasound for neonatal respiratory distress syndrome: a meta-analysis and systematic review. Med Ultrason. 22(3):325-33, 2020
                                                                      2. Magnani JE et al: Persistent respiratory distress in the term neonate: genetic surfactant deficiency diseases. Curr Pediatr Rev. 16(1):17-25, 2020
                                                                      3. Wu J et al: Lung ultrasound for the diagnosis of neonatal respiratory distress syndrome: a meta-analysis. Ultrasound Q. 36(2):102-10, 2020
                                                                      4. Corsini I et al: Lung ultrasound for the differential diagnosis of respiratory distress in neonates. Neonatology. 115(1):77-84, 2019
                                                                      5. Grimaldi C et al: Thoracic ultrasound accuracy for the investigation of initial neonatal respiratory distress. Arch Pediatr. 26(8):459-65, 2019
                                                                      6. Somaschini M et al: Surfactant proteins gene variants in premature newborn infants with severe respiratory distress syndrome. J Perinatol. 38(4):337-44, 2018
                                                                      7. Chen SW et al: Routine application of lung ultrasonography in the neonatal intensive care unit. Medicine (Baltimore). 96(2):e5826, 2017
                                                                      8. Hiles M et al: Neonatal respiratory distress syndrome: chest X-ray or lung ultrasound? A systematic review. Ultrasound. 25(2):80-91, 2017
                                                                      9. Kröner C et al: Lung disease caused by ABCA3 mutations. Thorax. 72(3):213-20, 2017
                                                                      10. Sardesai S et al: Evolution of surfactant therapy for respiratory distress syndrome: past, present, and future. Pediatr Res. 81(1-2):240-8, 2017