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Idiopathic infantile arterial calcification in two siblings: failure of treatment with diphosphonate

G Stuart1, C Wren, H Bain

  • 1Department of Paediatric Cardiology, Freeman Hospital, Newcastle upon Tyne.

British Heart Journal
|August 1, 1990
PubMed

Insights

Idiopathic infantile arterial calcification in siblings presents with fetal and postnatal echocardiographic abnormalities. Despite disodium etidronate treatment, both children with this rare condition died.

Area of Science:

  • Pediatric Cardiology
  • Neonatology
  • Genetics

Background:

  • Idiopathic infantile arterial calcification (IIAC) is a rare, severe condition affecting infants.
  • Early detection and understanding of IIAC are crucial for potential interventions.

Observation:

  • Two siblings presented with IIAC, exhibiting significant fetal and postnatal echocardiographic findings.
  • Key observations included large pericardial effusion, thickened valves, and great vessel calcification.

Findings:

  • Calcification was evident prenatally, detected as early as 33 weeks' gestation in one sibling.
  • Echocardiography revealed poor descending aorta pulsation and widespread arterial calcification.

Implications:

  • This case highlights the severe progression of IIAC even with early detection.
  • Further research into the genetic basis and novel therapeutic strategies for IIAC is warranted.

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