title: Current concepts in odontohypophosphatasia form of hypophosphatasia and report of two cases.
abstract: BACKGROUND
  Hypophosphatasia is a rare inherited disease derived from mutations in tissue non-specific alkaline phosphatase genes, with typical oral symptoms including short root anomaly and dysplasia of dentin or cementum.
  
  
  METHODS
  Two young female patients presented with short root anomaly with a history of premature loss of deciduous and/or permanent teeth. The laboratory and imaging investigations were performed. One case was diagnosed as odontohypophosphatasia concurrent with hyperthyroidism, the other was odontohypophosphatasia concurrent with multiple radicular cysts.
  
  
  CONCLUSIONS
  This report presents two cases of odontohypophosphatasia, a rare disease which is difficult to be diagnosed, and highlights that the history of premature loss of deciduous and/or permanent teeth, oral manifestation and laboratory tests are crucial for clinical diagnosis.
authors.affiliation: Department of Endodontics, Guanghua School of Stomatology, Sun Yat-sen University, Guangzhou, China.
authors.forename: Zhu-Yu
authors.initials: ZY
authors.lastname: Wang
authors.affiliation: Department of Endodontics, Guanghua School of Stomatology, Sun Yat-sen University, Guangzhou, China.
authors.forename: Kai
authors.initials: K
authors.lastname: Zhang
authors.affiliation: Department of Oral and Maxillofacial Surgery, Guanghua School of Stomatology, Sun Yat-sen University, Guangzhou, China.
authors.forename: Guang-Sen
authors.initials: GS
authors.lastname: Zheng
authors.affiliation: Department of Endodontics, Guanghua School of Stomatology, Sun Yat-sen University, Guangzhou, China.
authors.forename: Wei
authors.initials: W
authors.lastname: Qiao
authors.affiliation: Division of Oral and Maxillofacial Surgery, Faculty of Dentistry, the University of Hong Kong, 34 Hospital Road, Hong Kong, Hong Kong. richsu@hku.hk.
authors.forename: Yu-Xiong
authors.initials: YX
authors.lastname: Su
mesh_terms: D000469:Alkaline Phosphatase; D005260:Female; D006801:Humans; D007014:Hypophosphatasia; D009154:Mutation; D017001:Tooth Demineralization / Q000151:congenital*
chemical_list: D000469:Alkaline Phosphatase
keywords: Alkaline phosphatase; Cone-beam computed tomography; Dysplasia; Hyperthyroidism; Radicular cyst; Short root anomaly