Idiopathic Infantile Arterial Calcification: A Rare Cause of Sudden Unexpected Death in Childhood

Guimarães, Susana; Lopes, José Manuel; Bessa Oliveira, José; Santos, Agostinho
January 2010
Pathology Research International;2010, p1
Academic Journal
Case Study
Unexpected child death investigation is a difficult area of forensic practice in view of the wide range of possible genetic, congenital, and acquired natural and nonnatural causes. Idiopathic infantile arterial calcification (IIAC) is a rare autosomic recessive disease usually diagnosed postmortem. Inactivatingmutations of the ENPP1 gene were described in 80% of the cases with IIAC.We report a case of a 5-year-old girl submitted to a forensic autopsy due to sudden death and possible medical negligence/parents child abuse. Major alterations found (intimal proliferation and deposition of calcium hydroxyapatite around the internal elastic lamina and media of arteries; acute myocardial infarct, stenotic and calcified coronary artery; perivascular and interstitial myocardial fibrosis; and subendocardial fibroelastosis) were diagnostic of IIAC. We reviewed IIAC cases published in the English literature and highlight the importance of adequate autopsy evaluation in cases of sudden child death.


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