[Syndromes 4. Sanfilippo syndrome].

The Sanfilippo syndrome belongs to the mucopolysaccharidoses. In this syndrome heparansulfate accumulates in the lysosomes because of an enzymatic disorder in the degradation of mucopolysaccharides. The result is a function disorder of the involved tissues. The Sanfilippo syndrome is characterized by a combination of mental deterioration from the third year of life, hepatosplenomegaly and a typical facial appearance. These characteristics and the implications for dental treatment are discussed.