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DOI: 10.1007/s40556-018-0176-0
Idiopathic Arterial Calcification: Experience from a Single Center in South India
Abstract
Idiopathic arterial calcification of infancy is a rare autosomal recessive disease, characterized by deposition of calcium along the internal elastic membrane of arteries, accompanied by fibrous thickening of the intima which causes luminal narrowing. We hereby report a series of 20 cases seen over a period of 10 years. Nineteen cases were reported as idiopathic arterial calcification and one was reported as arterial calcification in a twin reversed arterial perfusion sequence. The sites of calcification were mainly aortic valve and root of aorta which was involved in 100% of cases followed by pulmonary artery up to its branches in 18 (90%) cases. There was recurrence in the subsequent pregnancies in three of the cases. Genetic association with ENPP1 was proved in one case. There was no survival in all the cases. Thus, the prognosis of idiopathic arterial calcification is very poor with very few long-term survivors.
Publication History
Received: 09 February 2018
Accepted: 11 May 2018
Article published online:
08 May 2023
© 2018. Society of Fetal Medicine. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)
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