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Surfactant Deficiency Disease
Hank Baskin, MD
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KEY FACTS

  • Terminology

    • Imaging

      • 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 about 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)
              • Rare genetic causes of surfactant dysfunction
              • Abnormal gene products adversely affect composition &/or function of surfactant
              • Most well-known causes: Surfactant protein-B, surfactant protein-C, ABCA3, & TTF-1 deficiencies
              • Often termed "surfactant dysfunction disorders"

          IMAGING

          • General Features

            • Radiographic Findings

              • CT Findings

                • Other Modality Findings

                  • Imaging Recommendations

                    • Ultrasonographic Findings

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          CLINICAL ISSUES

                          • Presentation

                            • Demographics

                              • Natural History & Prognosis

                                • Treatment

                                  Selected References

                                  1. Bener A: Psychological distress among postpartum mothers of preterm infants and associated factors: a neglected public health problem. Rev Bras Psiquiatr. 35(3):231-6, 2013
                                  2. Thavagnanam S et al: Variable clinical outcome of ABCA3 deficiency in two siblings. Pediatr Pulmonol. 48(10):1035-8, 2013
                                  3. Kleinlein B et al: Fatal neonatal respiratory failure in an infant with congenital hypothyroidism due to haploinsufficiency of the NKX2-1 gene: alteration of pulmonary surfactant homeostasis. Arch Dis Child Fetal Neonatal Ed. 96(6):F453-6, 2011
                                  4. Peca D et al: Altered surfactant homeostasis and recurrent respiratory failure secondary to TTF-1 nuclear targeting defect. Respir Res. 12:115, 2011
                                  5. Copetti R et al: Lung ultrasound in respiratory distress syndrome: a useful tool for early diagnosis. Neonatology. 94(1):52-9, 2008
                                  6. Kuhn JP et al: Caffey's Pediatric Diagnostic Imaging. 10th ed, vol I. Philadelphia: Mosby. 78-88, 2004
                                  7. Yurdakök M: Inherited disorders of neonatal lung diseases. Turk J Pediatr. 46(2):105-14, 2004
                                  8. Lemons JA et al: Very low birth weight outcomes of the National Institute of Child health and human development neonatal research network, January 1995 through December 1996. NICHD Neonatal Research Network. Pediatrics. 107(1):E1, 2001
                                  9. Newman B et al: Congenital surfactant protein B deficiency--emphasis on imaging. Pediatr Radiol. 31(5):327-31, 2001
                                  10. Whitsett JA et al: Acute respiratory disorders in neonatology. In Avery GB, Fletcher MA, MacDonald MG: Pathophysiology & Management of the Newborn. 5th ed. Philadelphia: Lippincott. 485, 1999
                                  11. Kossel H et al: 25 years of respiratory support of newborn infants. J Perinat Med. 25(5):421-32, 1997
                                  12. Swischuk LE et al: Immature lung problems: can our nomenclature be more specific? AJR Am J Roentgenol. 166(4):917-8, 1996
                                  13. Cleveland RH: A radiologic update on medical diseases of the newborn chest. Pediatr Radiol. 25(8):631-7, 1995
                                  Related Anatomy
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                                  Related Differential Diagnoses
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                                  References
                                  Tables

                                  Tables

                                  KEY FACTS

                                  • Terminology

                                    • Imaging

                                      • 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 about 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)
                                              • Rare genetic causes of surfactant dysfunction
                                              • Abnormal gene products adversely affect composition &/or function of surfactant
                                              • Most well-known causes: Surfactant protein-B, surfactant protein-C, ABCA3, & TTF-1 deficiencies
                                              • Often termed "surfactant dysfunction disorders"

                                          IMAGING

                                          • General Features

                                            • Radiographic Findings

                                              • CT Findings

                                                • Other Modality Findings

                                                  • Imaging Recommendations

                                                    • Ultrasonographic Findings

                                                      DIFFERENTIAL DIAGNOSIS

                                                        PATHOLOGY

                                                        • General Features

                                                          CLINICAL ISSUES

                                                          • Presentation

                                                            • Demographics

                                                              • Natural History & Prognosis

                                                                • Treatment

                                                                  Selected References

                                                                  1. Bener A: Psychological distress among postpartum mothers of preterm infants and associated factors: a neglected public health problem. Rev Bras Psiquiatr. 35(3):231-6, 2013
                                                                  2. Thavagnanam S et al: Variable clinical outcome of ABCA3 deficiency in two siblings. Pediatr Pulmonol. 48(10):1035-8, 2013
                                                                  3. Kleinlein B et al: Fatal neonatal respiratory failure in an infant with congenital hypothyroidism due to haploinsufficiency of the NKX2-1 gene: alteration of pulmonary surfactant homeostasis. Arch Dis Child Fetal Neonatal Ed. 96(6):F453-6, 2011
                                                                  4. Peca D et al: Altered surfactant homeostasis and recurrent respiratory failure secondary to TTF-1 nuclear targeting defect. Respir Res. 12:115, 2011
                                                                  5. Copetti R et al: Lung ultrasound in respiratory distress syndrome: a useful tool for early diagnosis. Neonatology. 94(1):52-9, 2008
                                                                  6. Kuhn JP et al: Caffey's Pediatric Diagnostic Imaging. 10th ed, vol I. Philadelphia: Mosby. 78-88, 2004
                                                                  7. Yurdakök M: Inherited disorders of neonatal lung diseases. Turk J Pediatr. 46(2):105-14, 2004
                                                                  8. Lemons JA et al: Very low birth weight outcomes of the National Institute of Child health and human development neonatal research network, January 1995 through December 1996. NICHD Neonatal Research Network. Pediatrics. 107(1):E1, 2001
                                                                  9. Newman B et al: Congenital surfactant protein B deficiency--emphasis on imaging. Pediatr Radiol. 31(5):327-31, 2001
                                                                  10. Whitsett JA et al: Acute respiratory disorders in neonatology. In Avery GB, Fletcher MA, MacDonald MG: Pathophysiology & Management of the Newborn. 5th ed. Philadelphia: Lippincott. 485, 1999
                                                                  11. Kossel H et al: 25 years of respiratory support of newborn infants. J Perinat Med. 25(5):421-32, 1997
                                                                  12. Swischuk LE et al: Immature lung problems: can our nomenclature be more specific? AJR Am J Roentgenol. 166(4):917-8, 1996
                                                                  13. Cleveland RH: A radiologic update on medical diseases of the newborn chest. Pediatr Radiol. 25(8):631-7, 1995