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[Diffuse arterial calcified elastopathy]
Insights
Diffuse arterial calcified elastopathy, a rare condition, was identified in pediatric patients with severe renovascular hypertension. This finding links arterial calcification to kidney issues in children, suggesting a new syndrome.
Area of Science:
- Vascular Medicine
- Pediatric Nephrology
- Radiology
- Pathology
Context:
- Severe renovascular hypertension in pediatric patients is a critical clinical challenge.
- Understanding the underlying causes of arterial abnormalities in children is essential for effective management.
- The 'dotted corticomedullary junction' on renal ultrasound is a novel imaging finding.
Purpose:
- To describe a clinicopathological syndrome of diffuse arterial calcified elastopathy in pediatric patients.
- To correlate imaging findings (renal ultrasonography) with histopathological evidence of arterial calcification.
- To investigate potential etiologies for this syndrome, including pseudoxanthoma elasticum.
Summary:
- Six pediatric patients with severe renovascular hypertension exhibited diffuse arterial calcified elastopathy.
- Renal ultrasonography revealed a characteristic 'dotted corticomedullary junction' due to calcified interlobar/arcuate arteries.
- Superficial temporal artery biopsies confirmed calcification within the elastic layers of muscular arteries; pseudoxanthoma elasticum was identified in two cases.
Impact:
- Identifies a distinct clinicopathological syndrome in pediatric renovascular hypertension.
- Highlights the utility of renal ultrasonography in diagnosing arterial calcification.
- Suggests potential etiological heterogeneity, necessitating further research into the causes of pediatric arterial calcification.
Abstract:
Diffuse arterial calcified elastopathy was observed in 6 pediatric patients presenting with severe renovascular hypertension. Renal ultrasonography showed a characteristic pattern, the dotted corticomedullary junction, related to the increased echogenicity of the interlobar and/or arcuate arteries. Superficial temporal artery biopsy demonstrated the presence and the extension of the calcifying process involving the elastic layers of the muscular arteries. This clinicopathological syndrome may be heterogeneous from an etiological point of view: etiologic investigations led to the diagnosis of pseudoxanthoma elasticum in 2 patients; no etiology could be found in the others.