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Idiopathic infantile arterial calcification in siblings: radiologic diagnosis and successful treatment
Insights
Idiopathic infantile arterial calcification, a rare condition causing arterial calcification and early death, can be diagnosed using radiographs. This study highlights two siblings diagnosed radiologically, with successful diphosphonate therapy in one case.
Area of Science:
- Pediatric Cardiology
- Radiology
- Biochemistry
Background:
- Idiopathic infantile arterial calcification (IIAC) is a rare, fatal disorder characterized by widespread arterial calcification of unknown cause.
- Coronary artery occlusion is the primary cause of mortality in affected infants.
- Radiographic diagnosis has been reported in a subset of literature cases.
Abstract:
Idiopathic infantile arterial calcification is a disorder of unknown etiology manifested by widespread arterial calcification. This usually leads to early death from coronary artery occlusion. In 12 of the 75 cases in the literature, radiographs were taken and it was possible to make the diagnosis in them all. We present two patients, siblings, in whom the diagnosis was established radiologically. The nature of the calcium deposits was studied in one of the infants and proved to be calcium hydroxyapatite. Therapy with diphosphonate was apparently successful in the other child.