Hypophosphatasia and the importance of the general dental practitioner - a case series and discussion of upcoming treatments

British Dental Journal
C FeeneyS Barry

Abstract

Hypophosphatasia (HPP) is an inherited metabolic disorder that results in poorly mineralised bones and teeth. Clinical symptoms vary widely from mild dental anomalies to fatal fetal defects. The most common dental symptoms include exfoliation of the primary incisors before the age of three with little or no root resorption, large pulp chambers, alveolar bone loss and thin dentinal walls. There is generally minimal periodontal inflammation associated with the bony destruction and tooth loss. The general dental practitioner is usually the first clinician to spot signs of the milder forms of HPP. Patients diagnosed with dental symptoms in childhood can go on to develop significant morbidity in middle age with chronic bone pain and stress fractures of the long bones. The primary dental care clinician is the key to early diagnosis of such cases, whether they present in childhood or adulthood. Emerging enzyme replacement therapy has considerably changed the landscape of the disease, resulting in astonishing improvements in bone mineralisation and a significant reduction in mortality and morbidity. It is increasingly likely that primary and secondary care clinicians will treat patients with the severe forms of HPP, who would previousl...Continue Reading

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Citations

Mar 23, 2019·Head and Neck Pathology·John Lennon Silva CunhaRicardo Luiz Cavalcanti de Albuquerque-Júnior
Jan 23, 2021·International Journal of Molecular Sciences·Stephanie GraserFranz Jakob
Aug 29, 2021·Journal of Prosthodontics : Official Journal of the American College of Prosthodontists·Yundong YangHuijuan Xiao

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