Introduction: Arterial calcification of infancy is a rare autosomal recessive genetic disorder with extremely poor prognosis characterized by extensive calcification of internal elastic lamina and thickened of intimal tissue of large and medium sized arteries.
Case Report: We present the case of a newborn necropsy with hydrops and widespread cyanosis. The internal examination revealed heart enlargement due to occlusive calcification of the arteries.
Discussion: The aim of the present report is to provide a correlation of clinical and pathological features. An update of genetic diagnosis is provided.
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