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Pseudohypertension in a child with Williams syndrome
C Narasimhan1, T Alexander, S Krishnaswami
1Department of Cardiology, Christian Medical College Hospital, Vellore, South India.
Pediatric Cardiology
|March 1, 1993
Summary
Pseudohypertension, a rare condition in children, was observed in a 5-year-old with Williams syndrome. This unusual case highlights thickened arterial vessels causing falsely high blood pressure readings.
Area of Science:
- Pediatric Cardiology
- Genetics
- Vascular Biology
Background:
- Pseudohypertension is typically observed in elderly individuals.
- Williams syndrome is a genetic disorder characterized by distinct facial features, supravalvar aortic stenosis, and peripheral pulmonary artery stenosis.
Observation:
- A 5-year-old child presented with clinical features suggestive of Williams syndrome.
- Repeated non-invasive blood pressure measurements using appropriately sized cuffs yielded significantly elevated readings.
- Simultaneous intra-arterial blood pressure measurements confirmed normal vascular pressures.
Findings:
- The discrepancy between non-invasive and intra-arterial blood pressure readings confirmed pseudohypertension.
- The underlying cause of pseudohypertension in this case was identified as excessively thickened arterial vessels.
- This finding is unusual, particularly in a pediatric patient with Williams syndrome.
Implications:
- This case underscores the importance of considering pseudohypertension in pediatric patients, especially those with connective tissue abnormalities or genetic syndromes.
- Accurate blood pressure assessment in children with Williams syndrome may require intra-arterial measurements to avoid misdiagnosis and inappropriate treatment.
- Understanding the vascular pathology in pediatric pseudohypertension can inform future diagnostic and therapeutic strategies.