Abstract
Hypophosphatasia is a congenital disease characterized by deficiency of serum and tissue non-specific alkaline phosphatase activity. The disease occurs due to mutations in the liver/bone/kidney alkaline phosphatase gene. Six clinical forms of hypophosphatasia are recognized. Systemic symptoms of the disease are respiratory complications, premature craniosynostosis, widespread demineralization and rachitic changes in the metaphases, stress fractures, chondrocalcinosis and osteoarthropathy. Characteristic dental symptoms are premature deciduous teeth loss, premature exfoliation of fully rooted primary teeth, severe dental caries and alveolar bone loss. This clinical report describes the prosthetic rehabilitation of a twenty two year-old Turkish female patient with hypophosphatasia.
Highlights
Hypophosphatasia is a rare bone disorder characterized by low or zero levels of the serum and tissue non-specific alkaline phosphatase necessary for normal bone mineralization [1]
The disease occurs due to mutations in the liver/bone/kidney alkaline phosphatase gene encoding the tissue-nonspecific alkaline phosphatase (TNAP or tissue-nonspecific alkaline phosphatise (TNSALP)) [2,3]
In which form of hypophosphatasia the patient is should be considered before making a dental treatment planning
Summary
Hypophosphatasia is a rare bone disorder characterized by low or zero levels of the serum and tissue non-specific alkaline phosphatase necessary for normal bone mineralization [1]. Premature loss of deciduous teeth is common dental symptoms of this form [11]. When a detailed history is obtained, premature loss of their deciduous teeth will be common complaint [13,14]. Abnormalities of the skeletal system are uncommon in odontohypophosphatasia It is characterized by premature exfoliation of fully rooted primary teeth and severe dental caries. Case presentation A twenty two year-old Turkish female patient with hypophosphatasia was referred to Department of Prosthetic Dentistry in Karadeniz Technical University with poor oral hygiene, missing teeth and unaesthetic appearance. Alveolar bone loss and enlarged pulp chambers were seen in the radiographic examination (Figure 2, 3). Third month control and she was pleased with her new appearance (Figure 7) Her motivation of oral hygiene had been increased
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