Mucopolysaccharidoses — a literature review and dental clinical studies

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Damian Drążewski, Anna Surdacka

1/2018/XLVI s. 81–87
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Fraza do cytowania: Drążewski D., Surdacka A. Mucopolysaccharidoses — a literature review and dental clinical studies. Dental Forum. 2018;XLVI(1):81–87. DOI:

Introduction. Mucopolysaccharidoses (MPSs) belong to hereditary storage diseases characterized by the deposition of partially degraded glycosaminoglycans within the lysosomes. As a result of a shortage of strictly defined lysosomal enzymes necessary for the glycosaminoglycan catalase, structural and functional damage to cells, tissues and entire organs occurs. Patients with MPSs suffer from both physical and mental abnormalities, which combined with the difficult access to professionall dental care can lead to a number of changes in the oral cavity. Aim. Assessment of oral health in patients with MPSs and determination of their need for dental care. Material and Methods. The study included 30 people aged from 2 to 30 years with different types of Mucopolysaccharidoses. A clinical study evaluating the state of teeth, gums and oral hygiene was carried out. Results. Values ​​of decay indexes for permanent and deciduous teeth were respectively: PUW from 3 to 28 and puw from 4 to 20. GI state index took values ​​from 0.75 to 3.0, oral hygiene of PI from 0.7 to 3.0, and OHIs from 1 up to 6. Conclusions. Patients with MPSs are characterized by poor oral health, which depends on the level of MPSs, thus requiring specialized dental care.

Key words: mucopolysaccharidosis, oral hygiene, oral health.

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