Abstract
Glycogen storage diseases (GSDs) are rare genetic disorders of glycogen metabolism where the liver, kidneys, respiratory and cardiac muscles, as well as the immune and skeletal systems can be affected. Oral manifestations can also be present, but the specificity and frequency of these manifestations in the different forms of GSD are unknown. Analysis of a case series of 60 patients presenting four types of GSD (Ia, Ib, III, and IX) showed that the different types of GSDs have common and specific oral manifestations. In none of the GSD types studied, the prevalence of caries was higher than in the general population, especially in patients benefiting from current nutritional therapy, while in all GSD types the prevalence of delayed tooth eruption, agenesis, and tooth shape abnormalities was increased compared to the general population. Severe periodontitis prevalence was increased in patients with GSD Ib and neutropenia. Our results show that GSDs have oral manifestations and suggest some specificity depending on the type of GSDs.
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References
Ainamo J, Bay I (1975) Problems and proposals for recording gingivitis and plaque. Int Dent J 25:229–235
Avsar A (2007) dental findings in a child with glycogen storage disease type IA. Quintessence Int Berl Ger 38:e36–e40
Baccetti T, Pierleoni L, Filippi L et al (1994) Dental and craniofacial findings in a child affected by glycogen storage disease type III. J Clin Pediatr Dent 19:55–60
Barrett AP, Buckley DJ, Katelaris CH (1990) Oral complications in type 1B glycogen storage disease. Oral Surg Oral Med Oral Pathol 69:174–176
Bartoli A, Bossu M, Sfasciotti G, Polimeni A (2006) Glycogen storage disease type Ib: a paediatric case report. Eur J Paediatr Dent 7:192–198
Bernabé E, Vehkalahti MM, Sheiham A et al (2016) The shape of the dose-response relationship between sugars and caries in adults. J Dent Res 95:167–172
Bolaños MV, Moussa H, Manrique MC, Bolaños MJ (2003) Radiographic evaluation of third molar development in Spanish children and young people. Forensic Sci Int 133:212–219. https://doi.org/10.1016/S0379-0738(03)00037-9
Bratthall D (2005) Estimation of global DMFT for 12-year-olds in 2004. Int Dent J 55:370–372
Burt BA, Pai S (2001) Sugar consumption and caries risk: a systematic review. J Dent Educ 65:1017–1023
Chaussain-Miller C, Sinding C, Wolikow M et al (2003) Dental abnormalities in patients with familial hypophosphatemic vitamin D-resistant rickets: prevention by early treatment with 1-hydroxyvitamin D. J Pediatr 142:324–331. https://doi.org/10.1067/mpd.2003.119
Chou JY, Jun HS, Mansfield BC (2010) Glycogen storage disease type I and G6Pase-beta deficiency: etiology and therapy. Nat Rev Endocrinol 6:676–688. https://doi.org/10.1038/nrendo.2010.189
Cudzinowski L (1979) Von Gierke’s disease: report of case. ASDC J Dent Child 46:413–415
Deas DE, Mackey SA, McDonnell HT (2003) systemic disease and periodontitis: manifestations of neutrophil dysfunction. Periodontol 32:82–104
Demirjian A, Goldstein H, Tanner J (1973) A new system of dental age assessment. Hum Biol 45:211–227
Dougherty N, Gataletto MA (1995) Oral sequelae of chronic neutrophil defects: case report of a child with glycogen storage disease type 1b. Pediatr Dent 17:224–229
Eke PI, Page RC, Wei L et al (2012) Update of the case definitions for population-based surveillance of periodontitis. J Periodontol 83:1449–1454. https://doi.org/10.1902/jop.2012.110664
Farrington FH, Duncan LL, Roth KS (1995) Looking a gift horse in the mouth: effects of cornstarch therapy and other implications of glycogen storage disease on oral hygiene and dentition. Pediatr Dent 17:311–311
Froissart R, Piraud M, Boudjemline AM et al (2011) Glucose-6-phosphatase deficiency. Orphanet J Rare Dis 6:27
Haritha A, Jayakumar A (2011) syndromes as they relate to periodontal disease. Periodontol 56:65–86
Hornecker E (2005) Periodontal and mucosal findings in glycogen-storage disease type Ib: a 12-year case report. Periodontal Pract Today 2:39–48
Katz J, Shenkman Z, Sela M et al (1997) Oral manifestations and anesthesia considerations in a child with glycogen storage disease type 1b: case report. Pediatr Dent 19:123–126
Khocht A, Albandar JM (2014) Aggressive forms of periodontitis secondary to systemic disorders. Periodontol 2000 65:134–148. https://doi.org/10.1111/prd.12015
Kidd SA, Rademeyer C, Roberts GJ et al (2002) Dental disease indices and caries-related microflora in children with glycogen storage disease. Int J Paediatr Dent 12:8–13
Klein H, Palmer CE, Knutson JW (1938) Studies on dental caries: I. Dental status and dental needs of elementary school children. Public Health Rep 1896-1970:751–765
Klein OD, Oberoi S, Huysseune A et al (2013) Developmental disorders of the dentition: an update.. Am J Med Genet C Semin Med Genet 163:318–332. https://doi.org/10.1002/ajmg.c.31382
Koven NL, Clark MM, Cody CS et al (1986) Impaired chemotaxis and neutrophil (polymorphonuclear leukocyte) function in glycogenosis type IB. Pediatr Res 20:438
Lingstrom P, van Houte J, Kashket S (2000) Food starches and dental caries. Crit Rev Oral Biol Med Off Publ Am Assoc Oral Biol 11:366–380
Loevy HT, Matalon R, Rosenthal IM (1983) Delayed dental age in hepatorenal glycogen storage disease. J Am Dent Assoc 1939 107:944–946
Maber M, Liversidge HM, Hector MP (2006) Accuracy of age estimation of radiographic methods using developing teeth. Forensic Sci Int 159:S68–S73. https://doi.org/10.1016/j.forsciint.2006.02.019
Matern D, Seydewitz HH, Bali D et al (2002) Glycogen storage disease type I: diagnosis and phenotype/genotype correlation. Eur J Pediatr 161(Suppl 1):S10–S19. https://doi.org/10.1007/s00431-002-0998-5
Mobley CC (2003) Nutrition and dental caries. Dent Clin N Am 47:319–336. https://doi.org/10.1016/S0011-8532(02)00102-7
Mortellaro C, Garagiola U, Carbone V et al (2005) Unusual oral manifestations and evolution in glycogen storage disease type Ib. J Craniofac Surg 16:45–52
Nolla CM (1960) The development of permanent teeth. J Dent Child 27:254–266
O’Leary TJ, Drake RB, Naylor JE (1972) The plaque control record. J Periodontol 43:38. https://doi.org/10.1902/jop.1972.43.1.38
Polder BJ, Van’t Hof MA, Van der Linden FP, Kuijpers-Jagtman AM (2004) A meta-analysis of the prevalence of dental agenesis of permanent teeth. Community Dent Oral Epidemiol 32:217–226
Ralls SA, Marshall EC (1985) dental management of a patient with glycogen storage disease type I. J Am Dent Assoc 110:723–726
Shifman A, Chanannel I (1978) Prevalence of taurodontism found in radiographic dental examination of 1,200 young adult Israeli patients. Community Dent Oral Epidemiol 6:200–203
Suri L, Gagari E, Vastardis H (2004) Delayed tooth eruption: pathogenesis, diagnosis, and treatment. A literature review. Am J Orthod Dentofac Orthop 126:432–445. https://doi.org/10.1016/j.ajodo.2003.10.031
van der Velden U (2005) Purpose and problems of periodontal disease classification. Periodontol 2000(39):13–21. https://doi.org/10.1111/j.1600-0757.2005.00127.x
Vastardis H (2000) The genetics of human tooth agenesis: new discoveries for understanding dental anomalies. Am J Orthod Dentofac Orthop 117:0650–0656. https://doi.org/10.1067/mod.2000.103257
Walter J, Labrune PA, Laforet P (2016) The glycogen storage diseases and related disorders. In: Inborn metabolic diseases. Springer, Berlin, pp 121–137
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Communicated by: Jean-Marie Saudubray
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Biosse Duplan, M., Hubert, A., Le Norcy, E. et al. Dental and periodontal manifestations of glycogen storage diseases: a case series of 60 patients. J Inherit Metab Dis 41, 947–953 (2018). https://doi.org/10.1007/s10545-018-0182-3
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DOI: https://doi.org/10.1007/s10545-018-0182-3