Two children with insulin-dependent diabetes mellitus (IDDM) presented with diabetic ketoacidosis within 4 months of being diagnosed with Kawasaki disease (KD). Both patients were African American males younger than 4 years of age. Immune markers associated with the autoimmune cause of diabetes were not detectable in these patients at the time of diagnosis with IDDM. In 1 patient, the immune markers were repeated 6 months after diagnosis but remained negative. Both patients currently require ∼1 unit of insulin/kg/d. Occurrence of IDDM after KD is rare; we could find no such reports in the English literature. The cause of diabetes in these 2 patients remains elusive, as does the cause of KD.

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