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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.
Insights
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.
Abstract:
We describe two twin sisters in whom calcification of different arteries was detected in the first weeks of life. Transient renal insufficiency, arterial hypertension, and skeletal abnormalities were also observed. One child had anasarca and heart decompensation at birth. Prenatal infarction of one kidney had occurred in the same infant. A kidney biopsy showed calcium deposits in all the layers of the arteries. Most findings in these patients are compatible with idiopathic arterial calcification of infancy (IACI). Investigation of calcium and phosphorus metabolism revealed spontaneously receding hypercalciuria, increased intraerythrocytic calcium levels, and transient X-ray abnormalities of the long bones. Treatment initially consisted of biphosphonate and later, the calcium antagonist flunarizin. A progressive diminution of the arterial calcification was observed in the course of both treatments.