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
Foster BL, Ramnitz MS, Gafni RI, et al. Rare bone diseases and their dental, oral, and craniofacial manifestations. J Dent Res. 2014 Jul;93(suppl 7):7S-19S.
Rockman-Greenberg C. Hypophosphatasia. Pediatr Endocrinol Rev. 2013;10(suppl 2):380-388.
Whyte MP. Physiological role of alkaline phosphatase explored in hypophosphatasia. Ann N Y Acad Sci. 2010;1192:190-200.
Mornet E. Hypophosphatasia. Best Pract Res Clin Rheumatol. 2008;22(1):113-127.
Mornet E, Nunes ME. Hypophosphatasia. In: Pagon RA, Adam MP, Ardinger HH, et al, eds. GeneReviews. Seattle, WA: University of Washington, Seattle; 1993. http://www.ncbi.nlm.nih.gov/books/NBK1150/. Published November 20, 2007. Updated February 4, 2016. Accessed June 8, 2016.
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.
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.
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;75:229-39.
van den Bos T, Handoko G, Niehof A, et al. Cementum and dentin in hypophosphatasia. J Dent Res. 2005;84(11):1021-1025.