Familial occurrence of Kawasaki syndrome in North America

Abstract

Objective: To describe families with multiple members affected with Kawasaki syndrome (KS) to increase awareness of the familial occurrence of KS among practitioners who care for these patients.

Design: Retrospective review of medical records at 2 medical centers and data collection from remote KS families who contacted the KS Research Program at the University of California, San Diego.

Results: Eighteen families with multiple affected members were identified. There were 9 families with 2 affected siblings. In San Diego, 3 (0.7%) of 424 KS families had sibling cases. Nine families were identified with KS in 2 generations or in multiple affected members, yielding a total of 24 KS-affected children. No clear pattern of inheritance could be deduced from these pedigrees, and it is likely that multiple polymorphic alleles influence KS susceptibility.

Conclusion: Physicians should counsel affected families and make them aware of the potential increased risk of KS among family members.

Figure

The 9 complex Kawasaki syndrome (KS) pedigrees identified (families 10–18), yielding a total of 24 KS-affected children, 3 of whom died of coronary artery complications. Circles indicate females; squares, males; unshaded symbols, unaffected individuals; shaded symbols, individuals with KS; and crossed-out symbols, deceased individuals.