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Oral Health Status of Patients with Lysosomal Storage Diseases in Poland

Author

Listed:
  • Damian Drążewski

    (Department of Conservative Dentistry and Periodontology, Poznan University of Medical Sciences, 60-812 Poznan, Poland)

  • Małgorzata Grzymisławska

    (Department of Anatomy, Poznan University of Medical Sciences, 61-781 Poznan, Poland)

  • Katarzyna Korybalska

    (Department of Pathophysiology, Poznan University of Medical Sciences, 60-806 Poznan, Poland)

  • Natasza Czepulis

    (Department of Pathophysiology, Poznan University of Medical Sciences, 60-806 Poznan, Poland)

  • Marian Grzymisławski

    (Department of Metabolic Disease, Nutrition and Internal Medicine, Poznan University of Medical Sciences, 60-355 Poznan, Poland)

  • Janusz Witowski

    (Department of Pathophysiology, Poznan University of Medical Sciences, 60-806 Poznan, Poland)

  • Anna Surdacka

    (Department of Conservative Dentistry and Periodontology, Poznan University of Medical Sciences, 60-812 Poznan, Poland)

Abstract

Patients with lysosomal storage diseases (LSDs) suffer from physical and mental disabilities, which together with poor access to professional care may lead to impaired oral health. This cross-sectional case-control study characterized the status of oral health in patients with LSDs in Poland. Thirty-six children and young adults with various forms of LSDs were examined. The data were compared with those from age- and sex-matched healthy controls. Exemplary cases were presented to highlight typical problems in oral care associated with LSDs. When possible, saliva was collected and analyzed for total protein, inflammatory mediators, and antioxidant status. Generally, patients with LSDs had significantly higher prevalence of caries, inferior gingival status, and inadequate oral hygiene. The severity of oral health impairment in mucopolysaccaridoses, the most common LSD in Poland, was similar to that seen in patients with mannosidoses or Pompe disease. Saliva could be collected only from few less handicapped patients. In MPS, it did not appear to differ significantly from the controls, but in patients with Pompe disease it contained lower concentrations of vascular endothelial growth factor (VEGF) and monocyte chemoattractant protein-1 (MCP-1), but higher levels of tumor necrosis factor receptors 1 and 2 (TNF-R1, TNF-R2) and myeloperoxidase (MPO). In conclusion, Polish patients with LSDs have an inadequate level of oral hygiene and substantially deteriorated oral health.

Suggested Citation

  • Damian Drążewski & Małgorzata Grzymisławska & Katarzyna Korybalska & Natasza Czepulis & Marian Grzymisławski & Janusz Witowski & Anna Surdacka, 2017. "Oral Health Status of Patients with Lysosomal Storage Diseases in Poland," IJERPH, MDPI, vol. 14(3), pages 1-10, March.
  • Handle: RePEc:gam:jijerp:v:14:y:2017:i:3:p:281-:d:92631
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    Cited by:

    1. Sabrina Wiemann & Nicolas Frenzel Baudisch & Rainer A. Jordan & Johannes Kleinheinz & Marcel Hanisch, 2018. "Oral Symptoms and Oral Health-Related Quality of Life in People with Rare Diseases in Germany: A Cross-Sectional Study," IJERPH, MDPI, vol. 15(7), pages 1-10, July.

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