[Dental study of hypophosphatasic child]

Area of Science:

• Biochemistry
• Genetics
• Pediatric Dentistry

Background:

• Hypophosphatasia is an inherited metabolic disorder characterized by deficient tissue-nonspecific alkaline phosphatase activity.
• Dental anomalies, including premature tooth shedding, are recognized clinical manifestations of hypophosphatasia.

Observation:

• A pediatric case presented with premature loss of lower deciduous incisors, prompting suspicion of hypophosphatasia.
• Systemic evaluation revealed low serum alkaline phosphatase and hypercalcemia.
• Dental examination showed significant alveolar bone resorption and deciduous tooth instability.

Findings:

• Despite systemic indicators, carpal bone age showed no delay, and cephalography revealed no major cranial abnormalities.
• Pathohistological analysis of extracted teeth indicated minor root apex cement resorption and a dysgonic periodontium.
• The case demonstrated progressive instability and shedding of deciduous teeth over a 2.5-year observation period.

Implications:

• This case underscores the importance of considering hypophosphatasia in pediatric patients with unexplained premature tooth loss.
• Early diagnosis and management are crucial for mitigating systemic and dental complications.
• Further research into the dental and skeletal manifestations of hypophosphatasia is warranted.