Oral Health Status of Patients with Lysosomal Storage Diseases in Poland
Abstract
:1. Introduction
2. Methodology
3. Results
3.1. Oral Examination
3.2. Exemplary Cases
3.2.1. Case 1. A 17-Year Old Boy with MPS Type IIIA (Patient No. 8 in Table 1)
3.2.2. Case 2. A 24-Year Old Man with Alpha-Mannosidosis (Patient No. 31 in Table 1)
3.2.3. Case 3. A 17-Year Old Boy with Late-Onset Pompe Disease (Patient No. 25 in Table 1)
3.3. Saliva Analysis
4. Discussion
5. Conclusions
Author Contributions
Conflicts of Interests
References
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Patient No. | Sex | Age | Mental Retardation | The Disease (Eponym) | The Enzyme Lacking | Dentition | DMFT | dmft | PLI | OHI-s | GI | Saliva Sample |
---|---|---|---|---|---|---|---|---|---|---|---|---|
1 | M | 19 | N | Mucopolysaccharidosis I (Hurler syndrome) | Alpha-L-Iduronidase | P | 4 | - | 0.7 | 1 | 0.75 | N |
2 | M | 10 | Y | Mucopolysaccharidosis II (Hunter syndrome) | Iduronate sulphatase | P | 8 | - | 1.6 | 2 | 1.58 | Y |
3 | M | 10 | Y | P | 9 | - | 2.5 | 4 | 2.04 | Y | ||
4 | M | 30 | N | P | 21 | - | 3.0 | 6 | 3.00 | N | ||
5 | M | 7 | Y | M | 4 | 7 | 2.1 | 2 | 1.91 | N | ||
6 | M | 12 | Y | P | 9 | - | 2.0 | 2 | 1.75 | N | ||
7 | M | 10 | Y | P | 8 | - | 0.8 | 1 | 0.95 | N | ||
8 | M | 17 | N | Mucopolysaccharidosis IIIA (Sanfilippo syndrome A) | Heparan sulfamidase | P | 22 | - | 2.6 | - | 2.37 | N |
9 | F | 8 | Y | D | - | 20 | 1.8 | 2 | 1.58 | N | ||
10 | M | 8 | Y | P | 16 | - | 2.8 | 4 | 2.37 | N | ||
11 | F | 7 | Y | M | 8 | 4 | 1.7 | 2 | 1.54 | N | ||
12 | F | 13 | Y | P | 7 | - | 1.7 | 2 | 1.41 | N | ||
13 | F | 9 | Y | M | 4 | 4 | 1.8 | 2 | 1.30 | N | ||
14 | M | 10 | Y | Mucopolysaccharidosis IIIB (Sanfilippo syndrome A) | N-acetyl-alpha-D-glucosaminidase | M | 4 | 4 | 1.8 | 2 | 1.30 | N |
15 | F | 16 | Y | P | 9 | - | 2.0 | 2 | 1.91 | N | ||
16 | F | 14 | Y | P | 4 | - | 0.8 | 1 | 0.87 | N | ||
17 | F | 8 | Y | M | 4 | 8 | 1.8 | 2 | 1.54 | N | ||
18 | F | 12 | Y | P | 11 | - | 2.8 | 3 | 2.33 | N | ||
19 | M | 16 | Y | P | 8 | - | 1.7 | 3 | 1.87 | N | ||
20 | F | 25 | N | Mucopolysaccharidosis IVA (Sanfilippo syndrome B) | Galactose-6-sulfate sulfatase | P | 8 | - | 1.7 | 2 | 1.58 | Y |
21 | M | 10 | Y | M | 3 | 9 | 2.0 | 2 | 1.79 | N | ||
22 | M | 15 | Y | P | 7 | - | 2.7 | 4 | 2.54 | N | ||
23 | M | 30 | N | Mucopolysaccharidosis IVB (Morquio syndrome B) | Beta-galactosidase | P | 28 | - | 3.0 | 6 | 3.00 | N |
24 | M | 12 | Y | Mucopolysaccharidosis VI (Maroteaux-Lamy syndrome) | Aryl-sulfatase B | P | 3 | - | 0.9 | 1 | 0.87 | N |
25 | M | 17 | Y | Pompe Disease (Glycogen storage disease type II) | Acid alpha-glucosidase | P | 8 | - | 2.8 | 4 | 2.75 | Y |
26 | F | 14 | Y | P | 10 | - | 2.7 | 4 | 2.84 | Y | ||
27 | M | 12 | Y | P | 14 | - | 3.0 | 4 | 2.90 | Y | ||
28 | F | 23 | Y | Alpha-mannosidosis | Alpha-D-mannosidase | P | 14 | - | 3.0 | 5 | 2.85 | Y |
29 | M | 22 | Y | P | 14 | - | 3.0 | 5 | 3.00 | Y | ||
30 | F | 18 | Y | P | 15 | - | 2.8 | 5 | 2.94 | Y | ||
31 | M | 24 | Y | P | 17 | - | 3.0 | 5 | 3.00 | N | ||
32 | M | 29 | Y | Niemann-Pick disease | Acid-sphingo-myelinase | P | 16 | - | 3.0 | 4 | 3.00 | N |
33 | M | 14 | Y | P | 10 | - | 3.0 | 2 | 3.00 | N | ||
34 | M | 24 | Y | Gangliosidosis I | Beta-galactosidase | P | 14 | - | 3.0 | 5 | 2.85 | Y |
35 | M | 11 | Y | Mucolipidosis II (Inclucion-cell disease) | Phosphotransfe-rase | M | 5 | 6 | 3.0 | 4 | 3.00 | Y |
36 | F | 24 | Y | Metachromatic leukodystrophy (MLD) | Aryl-sulfatase A | P | 9 | - | 1.8 | 3 | 2.90 | Y |
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Drążewski, D.; Grzymisławska, M.; Korybalska, K.; Czepulis, N.; Grzymisławski, M.; Witowski, J.; Surdacka, A. Oral Health Status of Patients with Lysosomal Storage Diseases in Poland. Int. J. Environ. Res. Public Health 2017, 14, 281. https://doi.org/10.3390/ijerph14030281
Drążewski D, Grzymisławska M, Korybalska K, Czepulis N, Grzymisławski M, Witowski J, Surdacka A. Oral Health Status of Patients with Lysosomal Storage Diseases in Poland. International Journal of Environmental Research and Public Health. 2017; 14(3):281. https://doi.org/10.3390/ijerph14030281
Chicago/Turabian StyleDrążewski, Damian, Małgorzata Grzymisławska, Katarzyna Korybalska, Natasza Czepulis, Marian Grzymisławski, Janusz Witowski, and Anna Surdacka. 2017. "Oral Health Status of Patients with Lysosomal Storage Diseases in Poland" International Journal of Environmental Research and Public Health 14, no. 3: 281. https://doi.org/10.3390/ijerph14030281