471 - A Case Report of Hurler Syndrome

Hurler syndrome (HS), is a rare genetic lysosomal storage disorder. Lysosomes contain enzymes that break down molecules such as fats and sugars in the body. HS is caused by a variation the gene for the protein that helps lysosomes break down complex sugars. Consequently, undigested sugar molecules accumulate and cause damage across multiple systems including the brain, heart, and vital organs. HS is inherited in an autosomal recessive pattern and is considered a pediatric syndrome because as the life expectancy of these patients only extends into the late teens and early twenties. Treatment of HS requires multidisciplinary collaboration with significant emphasis on dentistry due to the phenotypic presentation of patients which includes several dental-alveolar anomalies.