[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.
Visser
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Afdeling Mondziekten, Kaakchirurgie en Bijzondere Tandheelkunde, Academisch Ziekenhuis Groningen, postbus 30.001, 9700 RB Groningen.
Vissink
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D019465:Craniofacial Abnormalities / Q000209:etiology; D003813:Dentistry; D003937:Diagnosis, Differential; D006801:Humans; D009084:Mucopolysaccharidosis III / Q000175:diagnosis* / Q000473:pathology
Syndromen 4. Het Sanfilippo-syndroom.