Abstract
Idiopathic arterial calcification of infancy (IACI) is a rare disorder, characterized by extensive arterial calcification of large and medium sized arteries. We present two cases of IACI in siblings including the prenatal, postnatal and postmortem imaging, which is a hallmark of this disease.
Highlights
Idiopathic arterial calcification of infancy (IACI) is a rare disorder, characterized by extensive arterial calcification and stenoses of large and medium sized arteries
IACI is inherited in an autosomal recessive pattern
IACI was initially described by Bryant and White in 1901 [4]
Summary
Idiopathic arterial calcification of infancy (IACI) is a rare disorder, characterized by extensive arterial calcification and stenoses of large and medium sized arteries. Treatment with bisphosphonates, which are synthetic pyrophosphate analogues, has been proposed as a means of reducing arterial calcifications in IACI patients, but reported treatment regimens vary considerably both in terms of the specific bisphosphonate employed and the dose and duration of treatment [1,2,3]. This was the first baby born to non-consanguineous parents of mixed European descent at 37 weeks of gestation. The pericardial effusion resolved by 3 weeks of life She is 10 months old and remains asymptomatic, with normal growth and development.
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