Odontohypophosphatasia is a clinical form of hypophosphatasia primarily affecting the teeth.


(1) premature loss (before the age of 5) of primary/deciduous teeth with roots attached, starting with the incisors

(2) persistent primary teeth

(3) severe dental caries

(4) defective mineralization of teeth with potential for dental fractures

(5) alveolar bone loss

(6) mutation in the ALPL gene

(7) family members affected in a recessive or dominant pattern of inheritance

(8) persistent low serum alkaline phosphatase activity


Odontohypophosphatasia may occur:

(1) without skeletal manifestations of hypophosphatasia.

(2) in patients with juvenile or adult hypophosphatasia

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