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Idiopathic arterial calcification of infancy
P J Van Reempts1, K J Boven, S E Spitaels
1Department of Pediatrics, University Hospital of Antwerp, Antwerpen-Edegem, Belgium.
Calcified Tissue International
|January 1, 1991
Summary
Idiopathic arterial calcification of infancy (IACI) in twins presented with arterial calcification, renal issues, and hypertension. Treatments with biphosphonate and flunarizine showed progressive reduction in arterial calcification.
Area of Science:
- Pediatric Cardiology
- Neonatology
- Medical Genetics
Background:
- Idiopathic arterial calcification of infancy (IACI) is a rare, severe neonatal disorder.
- Characterized by widespread arterial calcification, leading to ischemic damage and potentially fatal outcomes.
- Early diagnosis and intervention are critical for managing IACI.
Observation:
- Two twin sisters presented with arterial calcification within weeks of birth.
- Observed complications included transient renal insufficiency, hypertension, and skeletal abnormalities.
- One twin exhibited congenital anasarca and heart decompensation, with prenatal renal infarction.
Findings:
- Kidney biopsy confirmed arterial calcification across all arterial layers.
- Metabolic investigations revealed hypercalciuria, elevated intraerythrocytic calcium, and bone abnormalities.
- Clinical presentation and findings were consistent with IACI.
Implications:
- This case highlights the presentation of IACI in twins.
- Successful management involved biphosphonate and calcium channel blockers, leading to reduced calcification.
- Further research into IACI's genetic and metabolic underpinnings is warranted.