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Idiopathic Infantile Arterial Calcification
Anne Kennedy, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Idiopathic infantile arterial calcification (IIAC)
              • Synonyms

                • Generalized arterial calcification of infancy
              • Definitions

                • Part of clinical spectrum of ectopic calcification with multiorgan pathology
                • Considerable overlap with
                  • Pseudoxanthoma elasticum (PXE)
                  • Autosomal recessive hypophosphatemic rickets

              IMAGING

              • General Features

                • Ultrasonographic Findings

                  DIFFERENTIAL DIAGNOSIS

                    PATHOLOGY

                    • General Features

                      • Gross Pathologic & Surgical Features

                        CLINICAL ISSUES

                        • Presentation

                          • Natural History & Prognosis

                            • Treatment

                              DIAGNOSTIC CHECKLIST

                              • Image Interpretation Pearls

                                Selected References

                                1. Boyce AM et al: Generalized arterial calcification of infancy: new insights, controversies, and approach to management. Curr Osteoporos Rep. 18(3):232-41, 2020
                                2. Dursun F et al: Magnesium and anti-phosphate treatment with bisphosphonates for generalised arterial calcification of infancy: a case report J Clin Res Pediatr Endocrinol. 11(3):311-8, 2019
                                3. Akhtar Ali S et al: Bisphosphonate therapy in an infant with generalized arterial calcification with an ABCC6 mutation. Osteoporos Int. 29(11):2575-9, 2018
                                4. Khan T et al: ENPP1 enzyme replacement therapy improves blood pressure and cardiovascular function in a mouse model of generalized arterial calcification of infancy. Dis Model Mech. 11(10), 2018
                                5. Yi Y et al: Prenatal diagnosis of idiopathic infantile arterial calcification without fetal hydrops. Echocardiography. 34(2):311-4, 2017
                                6. Li Q et al: Mutations in the ABCC6 gene as a cause of generalized arterial calcification of infancy: genotypic overlap with pseudoxanthoma elasticum. J Invest Dermatol. 134(3):658-65, 2014
                                7. Karthikeyan G: Generalized arterial calcification of infancy. J Pediatr. 162(5):1074.e1, 2013
                                8. Iglesias-Platas I et al: Two new cases of idiopathic arterial calcification in the newborn: watch out for lineal calcifications in plain radiographs. J Pediatr. 161(4):767-767.e1, 2012
                                9. Cansu A et al: Idiopathic infantile arterial calcification: prenatal diagnosis and postnatal presentation. Clin Exp Obstet Gynecol. 37(1):73-5, 2010
                                10. Heuser CC et al: Radiographic and pathologic evaluation of idiopathic infantile arterial calcification. Obstet Gynecol. 115(2 Pt 2):465-8, 2010
                                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

                                          • Diagnostic Checklist

                                            TERMINOLOGY

                                            • Abbreviations

                                              • Idiopathic infantile arterial calcification (IIAC)
                                            • Synonyms

                                              • Generalized arterial calcification of infancy
                                            • Definitions

                                              • Part of clinical spectrum of ectopic calcification with multiorgan pathology
                                              • Considerable overlap with
                                                • Pseudoxanthoma elasticum (PXE)
                                                • Autosomal recessive hypophosphatemic rickets

                                            IMAGING

                                            • General Features

                                              • Ultrasonographic Findings

                                                DIFFERENTIAL DIAGNOSIS

                                                  PATHOLOGY

                                                  • General Features

                                                    • Gross Pathologic & Surgical Features

                                                      CLINICAL ISSUES

                                                      • Presentation

                                                        • Natural History & Prognosis

                                                          • Treatment

                                                            DIAGNOSTIC CHECKLIST

                                                            • Image Interpretation Pearls

                                                              Selected References

                                                              1. Boyce AM et al: Generalized arterial calcification of infancy: new insights, controversies, and approach to management. Curr Osteoporos Rep. 18(3):232-41, 2020
                                                              2. Dursun F et al: Magnesium and anti-phosphate treatment with bisphosphonates for generalised arterial calcification of infancy: a case report J Clin Res Pediatr Endocrinol. 11(3):311-8, 2019
                                                              3. Akhtar Ali S et al: Bisphosphonate therapy in an infant with generalized arterial calcification with an ABCC6 mutation. Osteoporos Int. 29(11):2575-9, 2018
                                                              4. Khan T et al: ENPP1 enzyme replacement therapy improves blood pressure and cardiovascular function in a mouse model of generalized arterial calcification of infancy. Dis Model Mech. 11(10), 2018
                                                              5. Yi Y et al: Prenatal diagnosis of idiopathic infantile arterial calcification without fetal hydrops. Echocardiography. 34(2):311-4, 2017
                                                              6. Li Q et al: Mutations in the ABCC6 gene as a cause of generalized arterial calcification of infancy: genotypic overlap with pseudoxanthoma elasticum. J Invest Dermatol. 134(3):658-65, 2014
                                                              7. Karthikeyan G: Generalized arterial calcification of infancy. J Pediatr. 162(5):1074.e1, 2013
                                                              8. Iglesias-Platas I et al: Two new cases of idiopathic arterial calcification in the newborn: watch out for lineal calcifications in plain radiographs. J Pediatr. 161(4):767-767.e1, 2012
                                                              9. Cansu A et al: Idiopathic infantile arterial calcification: prenatal diagnosis and postnatal presentation. Clin Exp Obstet Gynecol. 37(1):73-5, 2010
                                                              10. Heuser CC et al: Radiographic and pathologic evaluation of idiopathic infantile arterial calcification. Obstet Gynecol. 115(2 Pt 2):465-8, 2010