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Dental

In hypophosphatasia (HPP), premature tooth loss is a common presenting symptom.1-7

Image courtesy of Research Center, Shriners Hospitals for Children - St. Louis
Left: Incisor from patient with hypophosphatasia with root intact. Right: Incisor with root absorbed.

In hypophosphatasia, defective acellular root cementum results in compromised peridontal attachment.1,8,9 Patients may experience2-9:

Premature tooth loss—with the root intact—before the age of five
Nontraumatic tooth loss at any age
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References: 1. Foster BL, Ramnitz MS, Gafni RI, et al. Rare bone diseases and their dental, oral, and craniofacial manifestations. J Dent Res. 2014 Jul;93(7 Suppl):7S-19S. 2. Rockman-Greenberg C. Hypophosphatasia. Pediatr Endocrinol Rev. 2013;10(suppl 2):380-388. 3. Whyte MP. Physiological role of alkaline phosphatase explored in hypophosphatasia. Ann N Y Acad Sci. 2010;1192:190-200. 4. Mornet E. Hypophosphatasia. Best Pract Res Clin Rheumatol. 2008;22(1):113-127. 5. Mornet E, Nunes ME. Hypophosphatasia. In: Pagon RA, Bird TD, Dolan CR, Stephen K, eds. GeneReviews. Seattle, WA: University of Washington, Seattle; 1993. http://www.ncbi.nlm.nih.gov/books/NBK1150/. Published November 20, 2007. Updated August 5, 2010. Accessed March 27, 2015. 6. Reibel A, Maniére M-C, Clauss F, et al. Orodental phenotype and genotype findings in all subtypes of hypophosphatasia. Orphanet J Rare Dis. 2009;4:6. 7. Whyte MP, Murphy WA, Fallon MD. Adult hypophosphatasia with chondrocalcinosis and arthropathy: variable penetrance of hypophosphatasemia in a large Oklahoma kindred. Am J Med. 1982;72(4):631-641. 8. Whyte MP, Zhang F, Wenkert D, et al. Hypophosphatasia: validation and expansion of the clinical nosology for children from 25 years experience with 173 pediatric patients. Bone. 2015 Jun;75:229-39. 9. van den Bos T, Handoko G, Niehof A, et al. Cementum and dentin in hypophosphatasia. J Dent Res. 2005;84(11):1021-1025.