Idiopathic Arterial Calcification: Experience from a Single Center in South India

Idiopathic Arterial Calcification: Experience from a Single Center in South India 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. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Journal of Fetal Medicine Springer Journals

Idiopathic Arterial Calcification: Experience from a Single Center in South India

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Publisher
Springer Journals
Copyright
Copyright © 2018 by Society of Fetal Medicine
Subject
Medicine & Public Health; Maternal and Child Health; Diagnostic Radiology; Ultrasound; Obstetrics/Perinatology/Midwifery; Reproductive Medicine
ISSN
2348-1153
eISSN
2348-8859
D.O.I.
10.1007/s40556-018-0176-0
Publisher site
See Article on Publisher Site

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.

Journal

Journal of Fetal MedicineSpringer Journals

Published: May 29, 2018

References

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