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Pulmonary Interstitial Glycogenosis
Mantosh S. Rattan, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Pulmonary interstitial glycogenosis (PIG)
            • Synonyms

              • Infantile cellular interstitial pneumonitis (former name)
              • Neonatal pulmonary interstitial glycogen accumulation disorder
                • More accurate description as glycogenosis refers to group of inborn errors of metabolism unrelated to this disease
            • Definitions

              • Specific disorder of unknown etiology in childhood interstitial lung disease (chILD) classification system
              • Results from infiltration & expansion of alveolar interstitium/septa by glycogen-laden mesenchymal cells
              • 1st described in 2002, but pathogenesis & physiologic sequelae have yet to be entirely elucidated

            IMAGING

            • General Features

              • Radiographic Findings

                • CT Findings

                  • Imaging Recommendations

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • General Features

                        • Microscopic Features

                          CLINICAL ISSUES

                          • Presentation

                            • Demographics

                              • Natural History & Prognosis

                                • Treatment

                                  DIAGNOSTIC CHECKLIST

                                  • Image Interpretation Pearls

                                    • Reporting Tips

                                      Selected References

                                      1. Fan LL et al: Diffuse lung disease in biopsied children 2 to 18 years of age. Application of the chILD classification scheme. Ann Am Thorac Soc. 12(10):1498-505, 2015
                                      2. Jiskoot-Ermers ME et al: Irreversible respiratory failure in a full-term infant with features of pulmonary interstitial glycogenosis as well as bronchopulmonary dysplasia. AJP Rep. 5(2):e136-40, 2015
                                      3. Ricca R et al: Pulmonary interstitial glycogenosis within a discrete pulmonary lesion mimicking congenital pulmonary airway malformation. Journal of Pediatric Surgery Case Reports. 3(9):371-3, 2015
                                      4. Guillerman RP et al: Contemporary perspectives on pediatric diffuse lung disease. Radiol Clin North Am. 49(5):847-68, 2011
                                      5. Castillo M et al: Pulmonary interstitial glycogenosis in the setting of lung growth abnormality: radiographic and pathologic correlation. Pediatr Radiol. 40(9):1562-5, 2010
                                      6. Deterding RR: Infants and young children with children's interstitial lung disease. Pediatr Allergy Immunol Pulmonol. 23(1):25-31, 2010
                                      7. Deutsch GH et al: Pulmonary interstitial glycogenosis: words of caution. Pediatr Radiol. 40(9):1471-5, 2010
                                      8. Lanfranchi M et al: Pulmonary interstitial glycogenosis. Pediatr Radiol. 40(3):361-5, 2010
                                      9. Deutsch GH et al: Diffuse lung disease in young children: application of a novel classification scheme. Am J Respir Crit Care Med. 176(11):1120-8, 2007
                                      10. Onland W et al: Pulmonary interstitial glycogenosis in identical twins. Pediatr Pulmonol. 40(4):362-6, 2005
                                      11. Smets K et al: Neonatal pulmonary interstitial glycogen accumulation disorder. Eur J Pediatr. 163(7):408-9, 2004
                                      12. Canakis AM et al: Pulmonary interstitial glycogenosis: a new variant of neonatal interstitial lung disease. Am J Respir Crit Care Med. 165(11):1557-65, 2002
                                      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

                                                  • Pulmonary interstitial glycogenosis (PIG)
                                                • Synonyms

                                                  • Infantile cellular interstitial pneumonitis (former name)
                                                  • Neonatal pulmonary interstitial glycogen accumulation disorder
                                                    • More accurate description as glycogenosis refers to group of inborn errors of metabolism unrelated to this disease
                                                • Definitions

                                                  • Specific disorder of unknown etiology in childhood interstitial lung disease (chILD) classification system
                                                  • Results from infiltration & expansion of alveolar interstitium/septa by glycogen-laden mesenchymal cells
                                                  • 1st described in 2002, but pathogenesis & physiologic sequelae have yet to be entirely elucidated

                                                IMAGING

                                                • General Features

                                                  • Radiographic Findings

                                                    • CT Findings

                                                      • Imaging Recommendations

                                                        DIFFERENTIAL DIAGNOSIS

                                                          PATHOLOGY

                                                          • General Features

                                                            • Microscopic Features

                                                              CLINICAL ISSUES

                                                              • Presentation

                                                                • Demographics

                                                                  • Natural History & Prognosis

                                                                    • Treatment

                                                                      DIAGNOSTIC CHECKLIST

                                                                      • Image Interpretation Pearls

                                                                        • Reporting Tips

                                                                          Selected References

                                                                          1. Fan LL et al: Diffuse lung disease in biopsied children 2 to 18 years of age. Application of the chILD classification scheme. Ann Am Thorac Soc. 12(10):1498-505, 2015
                                                                          2. Jiskoot-Ermers ME et al: Irreversible respiratory failure in a full-term infant with features of pulmonary interstitial glycogenosis as well as bronchopulmonary dysplasia. AJP Rep. 5(2):e136-40, 2015
                                                                          3. Ricca R et al: Pulmonary interstitial glycogenosis within a discrete pulmonary lesion mimicking congenital pulmonary airway malformation. Journal of Pediatric Surgery Case Reports. 3(9):371-3, 2015
                                                                          4. Guillerman RP et al: Contemporary perspectives on pediatric diffuse lung disease. Radiol Clin North Am. 49(5):847-68, 2011
                                                                          5. Castillo M et al: Pulmonary interstitial glycogenosis in the setting of lung growth abnormality: radiographic and pathologic correlation. Pediatr Radiol. 40(9):1562-5, 2010
                                                                          6. Deterding RR: Infants and young children with children's interstitial lung disease. Pediatr Allergy Immunol Pulmonol. 23(1):25-31, 2010
                                                                          7. Deutsch GH et al: Pulmonary interstitial glycogenosis: words of caution. Pediatr Radiol. 40(9):1471-5, 2010
                                                                          8. Lanfranchi M et al: Pulmonary interstitial glycogenosis. Pediatr Radiol. 40(3):361-5, 2010
                                                                          9. Deutsch GH et al: Diffuse lung disease in young children: application of a novel classification scheme. Am J Respir Crit Care Med. 176(11):1120-8, 2007
                                                                          10. Onland W et al: Pulmonary interstitial glycogenosis in identical twins. Pediatr Pulmonol. 40(4):362-6, 2005
                                                                          11. Smets K et al: Neonatal pulmonary interstitial glycogen accumulation disorder. Eur J Pediatr. 163(7):408-9, 2004
                                                                          12. Canakis AM et al: Pulmonary interstitial glycogenosis: a new variant of neonatal interstitial lung disease. Am J Respir Crit Care Med. 165(11):1557-65, 2002