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Kawasaki Disease in a Patient With Williams Syndrome
Aleisha M Nabower1, Lois J Starr1,2, Jonathan Cramer1,2
1University of Nebraska Medical Center, Nebraska Medical Center Omaha, NE, USA.
Insights
Diagnosing Kawasaki disease in infants with Williams syndrome is challenging. Early diagnosis and repeat echocardiography are crucial for identifying giant coronary aneurysms in these high-risk infants.
Area of Science:
- Pediatric Cardiology
- Genetics
- Rheumatology
Background:
- Kawasaki disease (KD) is a leading cause of acquired heart disease in children.
- Infants are at higher risk for coronary artery aneurysms (CAA) due to diagnostic challenges.
- Williams syndrome (WS) is a genetic disorder associated with various cardiovascular anomalies.
Observation:
- A 3-month-old infant with Williams syndrome presented with rapidly developing giant coronary aneurysms.
- The patient's presentation mimicked incomplete Kawasaki disease, complicating diagnosis.
- Repeat echocardiography was essential for timely diagnosis.
Findings:
- Kawasaki disease in infants with Williams syndrome can lead to rapid development of giant coronary aneurysms.
- Incomplete Kawasaki disease poses diagnostic challenges, especially in infants with pre-existing cardiac conditions.
- Elastin abnormalities in Williams syndrome may increase susceptibility to severe coronary artery damage from Kawasaki disease.
Implications:
- Highlights the critical role of serial echocardiography in diagnosing incomplete Kawasaki disease in infants.
- Suggests that genetic factors in Williams syndrome may predispose to severe coronary artery complications from Kawasaki disease.
- Emphasizes the need for heightened vigilance for Kawasaki disease in infants with Williams syndrome and cardiac anomalies.
Abstract:
Kawasaki disease can be difficult to diagnose in infants, putting them at higher risk for developing coronary artery dilatation. It can be even more difficult to diagnose in the setting of preexisting cardiac anomalies such as those found in Williams syndrome. We present a case of a three-month-old male with Williams syndrome with rapidly developing giant coronary aneurysms due to Kawasaki disease. This case demonstrates the importance of repeat echocardiography in diagnosing incomplete Kawasaki disease in infants. We speculate that elastin changes, as present in Williams syndrome, may put affected children at higher risk for development of giant coronary arteries should they acquire Kawasaki disease.
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