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[Diffuse arterial calcified elastopathy].

C Antignac, M C Gubler, L Garel

    Archives Des Maladies Du Coeur Et Des Vaisseaux
    |October 1, 1985
    PubMed
    Summary
    This summary is machine-generated.

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    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.

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  • 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.