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Idiopathic infantile arterial calcification: clinical presentation, therapy and long-term follow-up

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Abstract

Idiopathic infantile arterial calcification (IIAC) is a rare disease characterised by extensive depositions of hydroxyapatite in the internal elastic lamina of medium-sized and large arteries, frequently accompanied by periarticular calcifications. We report on three patients with various presenting signs and symptoms. Diagnostic imaging techniques and therapy with bisphosphonates will be discussed. For the first time long-term follow-up of up to 25 years will be reported.

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References

  1. Eronen M, Pohjavuori M, Heikkila P (2001) Fatal outcome of two siblings with idiopathic arterial calcification of infancy diagnosed in utero. Pediatr Cardiol 22:167–169

    Article  PubMed  CAS  Google Scholar 

  2. Levine JC, Campbell J, Nadel A (2001) Prenatal diagnosis of idiopathic infantile arterial calcification. Circulation 103:325–326

    PubMed  CAS  Google Scholar 

  3. Maayan C, Peleg O, Eyal F, Mogle P, Rosenmann E, Bar Ziv J (1984) Idiopathic infantile arterial calcification: a case report and review of the literature. Eur J Pediatr 142:211–215

    Article  PubMed  CAS  Google Scholar 

  4. Mahboubi S, Glaser DL, Shore EM, Kaplan FS (2001) Fibrodysplasia ossificans progressiva. Pediatr Radiol 31:307–314

    Article  PubMed  CAS  Google Scholar 

  5. Marrott PK, Newcombe KD, Becroft DM, Friedlander DH (1984) Idiopathic infantile arterial calcification with survival to adult life. Pediatr Cardiol 5:119–122

    Article  PubMed  CAS  Google Scholar 

  6. Meradji M, de Villeneuve VH, Huber J, de Bruijn WC, Pearse RG (1978) Idiopathic infantile arterial calcification in siblings: radiologic diagnosis and successful treatment. J Pediatr 92:401–405

    Article  PubMed  CAS  Google Scholar 

  7. Rutsch F, Ruf N, Vaingankar S, Toliat MR, Suk A, Hohne W, Schauer G, Lehmann M, Roscioli T, Schnabel D, Epplen JT, Knisely A, Superti-Furga A, McGill J, Filippone M, Sinaiko AR, Vallance H, Hinrichs B, Smith W, Ferre M, Terkeltaub R, Nurnberg P (2003) Mutations in ENPP1 are associated with ‘idiopathic’ infantile arterial calcification. Nat Genet 34:379–381

    Article  PubMed  CAS  Google Scholar 

  8. Rutsch F, Vaingankar S, Johnson K, Goldfine I, Maddux B, Schauerte P, Kalhoff H, Sano K, Boisvert WA, Superti-Furga A, Terkeltaub R (2001) PC-1 nucleoside triphosphate pyrophosphohydrolase deficiency in idiopathic infantile arterial calcification. Am J Pathol 158:543–554

    PubMed  CAS  Google Scholar 

  9. Srivastava T, Alon US (2003) The role of bisphosphonates in diseases of childhood. Eur J Pediatr 162:735–751

    Article  PubMed  CAS  Google Scholar 

  10. Stuart G, Wren C, Bain H (1990) Idiopathic infantile arterial calcification in two siblings: failure of treatment with diphosphonate. Br Heart J 64:156–159

    Article  PubMed  CAS  Google Scholar 

  11. Thiaville A, Smets A, Clercx A, Perlmutter N (1994) Idiopathic infantile arterial calcification: a surviving patient with renal artery stenosis. Pediatr Radiol 24:506–508

    Article  PubMed  CAS  Google Scholar 

  12. Thomas P, Chandra M, Kahn E, McVicar M, Naidich J, LaCorte M (1990) Idiopathic arterial calcification of infancy: a case with prolonged survival. Pediatr Nephrol 4:233–235

    Article  PubMed  CAS  Google Scholar 

  13. Van Dyck M, Proesmans W, Van Hollebeke E, Marchal G, Moerman P (1989) Idiopathic infantile arterial calcification with cardiac, renal and central nervous system involvement. Eur J Pediatr 148:374–377

    Article  PubMed  Google Scholar 

  14. Vera J, Lucaya J, Garcia Conesa JA, Aso C, Balaguer A (1990) Idiopathic infantile arterial calcification: unusual features. Pediatr Radiol 20:585–587

    Article  PubMed  CAS  Google Scholar 

  15. Whitehall J, Smith M, Altamirano L (2003) Idiopathic infantile arterial calcification: sonographic findings. J Clin Ultrasound 31:497–501

    Article  PubMed  Google Scholar 

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Correspondence to Inge M. van der Sluis.

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van der Sluis, I.M., Boot, A.M., Vernooij, M. et al. Idiopathic infantile arterial calcification: clinical presentation, therapy and long-term follow-up. Eur J Pediatr 165, 590–593 (2006). https://doi.org/10.1007/s00431-006-0146-8

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  • DOI: https://doi.org/10.1007/s00431-006-0146-8

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