Idiopathic Infantile Arterial Calcification: A Case Report
Journal Title: Journal of Pediatrics & Child Care - Year 2017, Vol 3, Issue 1
Abstract
Idiopathic Infantile Arterial Calcification (IIAC) is a rare autosomal recessive inherited disorder that influences the metabolism of pyrophosphate and leads to in-utero calcium deposition with fibrotic proliferation in the walls of the large and medium-sized arteries. IIAC may present antenatally with polyhydramnios with or without arterial calcification, and may present postnatally if intractable systemic hypertension and/or heart failure from systemic hypertension and or ischemic coronary artery disease. Diagnosis of IIAC is usually confirmed by the genetic study. It has a poor prognosis, with mortality been reported by the age of 4-6 months. Increasing awareness about IIAC facilitates early diagnosis, early management, family counseling for prognosis and future conceptual plans, as early diagnosis helps in the decision of pregnancy termination.
Authors and Affiliations
Ghassan A Shaath
Keywords
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- EP ID EP246045
- DOI 10.13188/2380-0534.1000019
- Views 115
- Downloads 0
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