Bağiş Bora, Baltacioğlu Esra, Aydoğan Elif, Tamam Evşen
Assistant Professor, Department of Prosthodontics, Faculty of Dentistry, Karadeniz Technical University, Trabzon, Turkey.
Cases J. 2008 Dec 12;2:7626. doi: 10.1186/1757-1626-2-7626.
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.
低磷酸酯酶症是一种先天性疾病,其特征为血清和组织非特异性碱性磷酸酶活性缺乏。该疾病是由肝/骨/肾碱性磷酸酶基因突变引起的。已确认低磷酸酯酶症有六种临床类型。该疾病的全身症状包括呼吸并发症、过早颅缝早闭、广泛脱矿以及干骺端佝偻病样改变、应力性骨折、软骨钙质沉着症和骨关节炎。典型的牙齿症状包括乳牙过早脱落、完全萌出的乳牙过早脱落、严重龋齿和牙槽骨丧失。本临床报告描述了一名患有低磷酸酯酶症的22岁土耳其女性患者的假体康复情况。