PMID- 28282939 OWN - NLM STAT- MEDLINE DCOM- 20170731 LR - 20181202 IS - 1660-4601 (Electronic) IS - 1661-7827 (Print) IS - 1660-4601 (Linking) VI - 14 IP - 3 DP - 2017 Mar 9 TI - Oral Health Status of Patients with Lysosomal Storage Diseases in Poland. LID - 10.3390/ijerph14030281 [doi] LID - 281 AB - 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. FAU - Drazewski, Damian AU - Drazewski D AD - Department of Conservative Dentistry and Periodontology, Poznan University of Medical Sciences, 60-812 Poznan, Poland. damian.stom@gmail.com. FAU - Grzymislawska, Malgorzata AU - Grzymislawska M AD - Department of Anatomy, Poznan University of Medical Sciences, 61-781 Poznan, Poland. malgorzatagrzymislawska@ump.edu.pl. FAU - Korybalska, Katarzyna AU - Korybalska K AD - Department of Pathophysiology, Poznan University of Medical Sciences, 60-806 Poznan, Poland. koryb@amp.edu.pl. FAU - Czepulis, Natasza AU - Czepulis N AD - Department of Pathophysiology, Poznan University of Medical Sciences, 60-806 Poznan, Poland. czepulis@ump.edu.pl. FAU - Grzymislawski, Marian AU - Grzymislawski M AD - Department of Metabolic Disease, Nutrition and Internal Medicine, Poznan University of Medical Sciences, 60-355 Poznan, Poland. mariangrzym@ump.edu.pl. FAU - Witowski, Janusz AU - Witowski J AD - Department of Pathophysiology, Poznan University of Medical Sciences, 60-806 Poznan, Poland. jwitow@ump.edu.pl. FAU - Surdacka, Anna AU - Surdacka A AD - Department of Conservative Dentistry and Periodontology, Poznan University of Medical Sciences, 60-812 Poznan, Poland. annasurd@gmail.com. LA - eng PT - Journal Article DEP - 20170309 PL - Switzerland TA - Int J Environ Res Public Health JT - International journal of environmental research and public health JID - 101238455 SB - IM MH - Adolescent MH - Adult MH - Case-Control Studies MH - Child MH - Cross-Sectional Studies MH - Dental Caries/*etiology MH - Female MH - *Health Status MH - Humans MH - Lysosomal Storage Diseases/*complications/*epidemiology MH - Male MH - *Oral Health MH - Oral Hygiene MH - Poland/epidemiology MH - Prevalence MH - Saliva/*chemistry MH - Young Adult PMC - PMC5369117 OTO - NOTNLM OT - developmental disabilities OT - lysosomal storage disease OT - oral health OT - saliva EDAT- 2017/03/12 06:00 MHDA- 2017/08/02 06:00 PMCR- 2017/03/01 CRDT- 2017/03/12 06:00 PHST- 2017/01/16 00:00 [received] PHST- 2017/02/20 00:00 [revised] PHST- 2017/03/02 00:00 [accepted] PHST- 2017/03/12 06:00 [entrez] PHST- 2017/03/12 06:00 [pubmed] PHST- 2017/08/02 06:00 [medline] PHST- 2017/03/01 00:00 [pmc-release] AID - ijerph14030281 [pii] AID - ijerph-14-00281 [pii] AID - 10.3390/ijerph14030281 [doi] PST - epublish SO - Int J Environ Res Public Health. 2017 Mar 9;14(3):281. doi: 10.3390/ijerph14030281.