Journals / Turkish Journal of Pediatrics / 2018 / Cilt: 60 - Sayı: 4

Oral health status in patients with mucopolysaccharidoses

Pages
400–406
DOI
—
🏛️Bibliographic metadata and full text access for this article are provided via DergiPark.
DergiPark Record ↗
✨

AI Summary & Key Takeaways

AI Insight
⚡ TL;DR(Too Long; Didn't Read)

Regular dental counseling, preventive applications and dentaltreatments must be included in multidisciplinary team approach of MPSpatients to provide better oral health.

📌 3 Key Takeaways

🎯
Objective & Hypothesis:The aim of this study is to assess oral clinical and radiological findingsof patients with mucopolysaccharidoses (MPS) which are a group of rareinherited disorders of glycosaminoglycan catabolism.
📊
Key Findings & Data:In this retrospective anddescriptive study, records of all patients with MPS over 3 years of age managedat Division of Pediatric Metabolism, who were all referred to Department ofPediatric Dentistry for evaluation of their oral health status were assessed.Oral examinations were performed in 30 patients with determination ofDMFT/S, dmft/s, dental plaque and gingival indices and panoramic radiologicalassessment was performed in 12 patients.
💡
Conclusion & Impact:Regular dental counseling, preventive applications and dentaltreatments must be included in multidisciplinary team approach of MPSpatients to provide better oral health.
🔬Design:Research Article
📍Scope:Comprehensive Sample
⚙️Variables:Domain Parameters

Özet

The aim of this study is to assess oral clinical and radiological findingsof patients with mucopolysaccharidoses (MPS) which are a group of rareinherited disorders of glycosaminoglycan catabolism. In this retrospective anddescriptive study, records of all patients with MPS over 3 years of age managedat Division of Pediatric Metabolism, who were all referred to Department ofPediatric Dentistry for evaluation of their oral health status were assessed.Oral examinations were performed in 30 patients with determination ofDMFT/S, dmft/s, dental plaque and gingival indices and panoramic radiologicalassessment was performed in 12 patients. Twenty-seven (90.0%) patientshad dental caries and 19/21 (90.5%) had gingival inflammation. Radiologicalexamination in 12 patients with panoramic image showed short mandibularramus (100.0%), enlarged dental follicles (83.3%), taurodontism (75.0%),flat mandibular notch (66.7%). In association with the bone structure andmorphology of the jaws, 11 patients (91.7%) had trabecular variation similarto osteoporotic disease. Condylar defect was observed in all patients, whichmight lead to temporomandibular joint disorders. Pulp chamber with threehorns, which had never been reported in MPS before, was observed inpanoramic images of 2 of 7 patients with MPS-VI. Oral clinical and radiologicalchanges are very common in this population of MPS patients. These changesmay impair opening of the mouth, eating and chewing and thus affect theirquality of life. Regular dental counseling, preventive applications and dentaltreatments must be included in multidisciplinary team approach of MPSpatients to provide better oral health.

🔖

Cite This Article

BALLIKAYA, E., EYMİRLİ, P. S., YILDIZ, Y., AVCU, N., SİVRİ, H. S., TEKÇİÇEK, M. U. (2018). Oral health status in patients with mucopolysaccharidoses. Turkish Journal of Pediatrics, Cilt: 60 - Sayı: 4, 400–406. https://ajindex.com/articles/412160

📄 Full Text PDF Viewer

📄

PDF görüntüleyici hazırlanıyor...

References (13)

  1. Nelson J, Crowhurst J, Carey B, Greed L. Incidence of the mucopolysaccharidoses in Western Australia. Am J Med Genet A 2003; 123A: 310-313
  2. Lin HY, Lin SP, Chuang CK, et al. Incidence of the mucopolysaccharidoses in Taiwan, 1984-2004. Am J Med Genet A 2009; 149A: 960-964
  3. Baehner F, Schmiedeskamp C, Krummenauer F, et al. Cumulative incidence rates of the mucopolysaccharidoses in Germany. J Inherit Metab Dis 2005; 28: 1011-1017
  4. V i e i r a T, S c h w a r t z I, M u n o z V, e t a l. Mucopolysaccharidoses in Brazil: What happens from birth to biochemical diagnosis? Am J Med Genet A 2008; 146A: 1741-1747
  5. Meikle PJ, Hopwood JJ, Clague AE, Carey WF. Prevalence of lysosomal storage disorders. JAMA 1999; 281: 249-254
  6. Koc I. Prevalence and sociodemographic correlates of consanguineous marriages in Turkey. J Biosoc Sci 2008; 40: 137-148
  7. Muenzer J, Wraith JE, Clarke LA; International Consensus Panel on Management and Treatment of Mucopolysaccharidosis I. Mucopolysaccharidosis I: Management and treatment guidelines. Pediatrics 2009; 123: 19-29
  8. Antunes LAA, Nogueira APB, Castro GF, Ribeiro MG, de Souza IPR. Dental findings and oral health status in patients with mucopolysaccharidosis: A case series. Acta Odontol Scand 2013; 71: 157-167
  9. Ponciano S, Areias C, Leão-Teles E, Sampaio-Maia B. Hyposalivation, acidic saliva, decayed teeth and oral yeast prevalence in children with mucopolysaccharidosis. MedicalExpress 2015; 2: M150502 10. Muenzer J. The mucopolysaccharidoses: A heterogeneous group of disorders with variable pediatric presentations. J Pediatr 2004; 144 (Suppl 5): S27-S34. 11. de Almeida-Barros RQ, Oka SC, Pordeus AC, de Medeiros PF, Bento PM, Godoy GP. Oral and systemic manifestations of mucopolysaccharidosis type VI: A report of seven cases. Quintessence Int 2012; 43: e32-e38
  10. James A, Hendriksz CJ, Addison O. The oral health needs of children, adolescents and young adults affected by a mucopolysaccharide disorder. JIMD Rep 2012; 2: 51-58
  11. Alpöz AR, Coker M, Celen E, et al. The oral manifestations of Maroteaux-Lamy syndrome (mucopolysaccharidosis VI): A case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2006; 101: 632-637
  12. G u v e n G, C e h r e l i Z C, A l t u n C, e t a l. Mucopolysaccharidosis type I (Hurler syndrome): Oral and radiographic findings and ultrastructural/chemical features of enamel and dentin. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2008; 105: 72-78
  13. Kantaputra PN, K