Key result
Forensic autopsy reveals idiopathic infantile arterial calcification in a sudden child death.
Why the study?
Idiopathic infantile arterial calcification (IIAC) is a rare disease usually diagnosed postmortem and can cause sudden unexpected death in childhood, requiring detailed forensic investigation.
Case Report (n=1)
Idiopathic infantile arterial calcification is a rare but important cause of sudden unexpected death in childhood that can be diagnosed via thorough forensic autopsy.
Answers are grounded in this paper and cite their sources.
Rare arteriopathies may underlie unexplained pediatric sudden death; case reports leave open the need for standardized autopsy protocols.
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. Inactivating mutations 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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Guimarães et al. (2010) conducted a case report in Idiopathic infantile arterial calcification (IIAC) (n=1). Idiopathic infantile arterial calcification was evaluated on Autopsy findings diagnostic of IIAC. Forensic autopsy of a 5-year-old girl with sudden death revealed idiopathic infantile arterial calcification, highlighting the importance of adequate autopsy evaluation in sudden child death.
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