Idiopathic infantile arterial calcification natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ayokunle Olubaniyi, M.B,B.S [2]

Overview

Natural History, Complication and Prognosis

  • Although survival to adulthood has been reported,[1][2] sometimes with persistent hypertension and cardiovascular sequelae, most patients die within the first 6 months of life.
  • Spontaneous regression of arterial calcifications can occur, and antihypertensive treatment can be tapered off gradually. In some patients, the natural course of GACI may be more favourable than previously assumed.[3][4]
  • Despite the same genotype and similar sonographic and radiographic features in early infancy, the phenotype of IIAC can vary to a great extent within one family.[5]

References

  1. Sholler, Gary F.; Yu, John S.; Bale, Patricia M.; Hawker, Richard E.; Celermajer, John M.; Kozlowski, Kasimir (1984). "Generalized arterial calcification of infancy: Three case reports, including spontaneous regression with long-term survival". The Journal of Pediatrics. 105 (2): 257–60. doi:10.1016/S0022-3476(84)80123-7. PMID 6747757.
  2. Marrott, Pran K.; Newcombe, Ken D.; Becroft, David M. O.; Friedlander, Denis H. (1984). "Idiopathic infantile arterial calcification with survival to adult life". Pediatric Cardiology. 5 (2): 119–22. doi:10.1007/BF02424963. PMID 6473121.
  3. Ciana, Giovanni; Trappan, Antonella; Bembi, Bruno; Benettoni, Alessandra; Maso, Giampaolo; Zennaro, Floriana; Ruf, Nico; Schnabel, Dirk; Rutsch, Frank (2005). "Generalized arterial calcification of infancy: Two siblings with prolonged survival". European Journal of Pediatrics. 165 (4): 258–63. doi:10.1007/s00431-005-0035-6. PMID 16315058.
  4. Thomas, Philomena; Chandra, Manju; Kahn, Ellen; McVicar, Melinda; Naidich, James; Lacorte, Michael (1990). "Idiopathic arterial calcification of infancy: A case with prolonged survival". Pediatric Nephrology. 4 (3): 233–5. doi:10.1007/BF00857661. PMID 2400650.
  5. Cheng, Kun-Shan; Chen, Ming-Ren; Ruf, Nico; Lin, Shuan-Pei; Rutsch, Frank (2005). "Generalized arterial calcification of infancy: Different clinical courses in two affected siblings". American Journal of Medical Genetics Part A. 136A (2): 210–3. doi:10.1002/ajmg.a.30800.


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