Maple syrup urine disease is a rare inborn error of metabolism that manifests mainly by neurologic deterioration, coma, and death in early childhood. Oral manifestations described in this article were mostly gingival enlargement and rampant caries, while histopathologic findings revealed a picture compatible osteomyelitis.
Notes:
Gazit, D Zeltser, R Galili, D Kaufman, E eng Case Reports 1989/06/01 Oral Surg Oral Med Oral Pathol. 1989 Jun;67(6):694-7.
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