CHRONIC IDIOPATHIC HYPERPHOSPHATASEMIA - CASE-REPORT

Chronic idiopathic hyperphosphatasemia, or juvenile Paget disease is a very rare syndrome that is characterized by fragile bones, bowing def ormities, shortness of stature, large head, premature loss of teeth, r adiographic evidence of expanded osteoporotic long bones with coarse t rabeculations, and widened bones of the skull. Increased levels of ser um alkaline phosphatase and increased levels of urinary total hydroxyp roline are notable. We present a case of juvenile Paget disease that w as associated with a history of precocious puberty. The patient had od ontogenic osteomyelitis of the mandible that was treated by drainage, surgical debridement and antibiotic therapy.