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Hypertension as the major problem of idiopathic arterial calcification of infancy
Insights
Idiopathic arterial calcification of infancy can cause severe hypertension refractory to treatment. Management may involve renin antagonists and nephrectomy for renovascular disease, with diagnosis requiring high suspicion.
Area of Science:
- Pediatric Cardiology
- Nephrology
- Genetics
Background:
- Idiopathic arterial calcification of infancy (IAC) is a rare, severe disorder characterized by diffuse calcification of the media of large and small arteries.
- Severe hypertension is a critical and often life-threatening manifestation of IAC.
Observation:
- Two infants presented with severe hypertension secondary to IAC.
- Both infants exhibited resistance to conventional and aggressive antihypertensive medications.
Findings:
- Radiologic and laboratory findings indicated significant renovascular disease in both cases.
- Treatment with renin antagonists and subsequent nephrectomy led to a moderate reduction in blood pressure.
- Renal pathology confirmed renovascular compromise as a contributor to hypertension.
Implications:
- This case series highlights the complex management of hypertension in IAC.
- Early recognition and suspicion are crucial for timely diagnosis and intervention.
- Understanding the renovascular component is vital for optimizing therapeutic strategies in affected infants.
Abstract:
We describe two infants with severe hypertension associated with idiopathic arterial calcification of infancy. In both children, blood pressure control was refractory to aggressive antihypertensive therapy. There was radiologic and laboratory evidence of renovascular disease requiring the use of specific renin antagonists and later nephrectomy, resulting in moderate improvement of hypertension. Although on occasion there is a familial incidence, in the vast majority of cases the diagnosis hinges on a high index of suspicion.