Abstract：
BACKGROUND: Mineral metabolism is critical for proper development of hard tissues of the skeleton and dentition. The dentoalveolar complex includes the following 4 mineralized tissues: enamel, dentin, cementum, and alveolar bone. Developmental processes of these tissues are affected by inherited disorders that disrupt phosphate and pyrophosphate homeostasis, although manifestations are distinct from those in the skeleton.
METHODS: The authors discuss original data from experiments and comparative analyses and review articles describing effects of inherited phosphate and pyrophosphate disorders on dental tissues. A particular emphasis is placed on how new therapeutic approaches for these conditions may affect oral health and dental treatments of affected patients.
RESULTS: Disorders of phosphate and pyrophosphate metabolism can lead to reduced mineralization (hypomineralization) or inappropriate (ectopic) calcification of soft tissues. Disruptions in phosphate levels in X-linked hypophosphatemia and hyperphosphatemic familial tumoral calcinosis and disruptions in pyrophosphate levels in hypophosphatasia and generalized arterial calcification of infancy contribute to dental mineralization defects. Traditionally, there have been few options to ameliorate dental health problems arising from these conditions. New antibody and enzyme replacement therapies bring possibilities to improve oral health in affected patients.
CONCLUSIONS: Research over the past 2 decades has exponentially expanded the understanding of mineral metabolism, and has led to novel treatments for mineralization disorders. Newly implemented and emerging therapeutic strategies affect the dentoalveolar complex and interact with aspects of oral health care that must be considered for dental treatment, clinical trial design, and coordination of multidisciplinary care teams.
METHODS: The authors discuss original data from experiments and comparative analyses and review articles describing effects of inherited phosphate and pyrophosphate disorders on dental tissues. A particular emphasis is placed on how new therapeutic approaches for these conditions may affect oral health and dental treatments of affected patients.
RESULTS: Disorders of phosphate and pyrophosphate metabolism can lead to reduced mineralization (hypomineralization) or inappropriate (ectopic) calcification of soft tissues. Disruptions in phosphate levels in X-linked hypophosphatemia and hyperphosphatemic familial tumoral calcinosis and disruptions in pyrophosphate levels in hypophosphatasia and generalized arterial calcification of infancy contribute to dental mineralization defects. Traditionally, there have been few options to ameliorate dental health problems arising from these conditions. New antibody and enzyme replacement therapies bring possibilities to improve oral health in affected patients.
CONCLUSIONS: Research over the past 2 decades has exponentially expanded the understanding of mineral metabolism, and has led to novel treatments for mineralization disorders. Newly implemented and emerging therapeutic strategies affect the dentoalveolar complex and interact with aspects of oral health care that must be considered for dental treatment, clinical trial design, and coordination of multidisciplinary care teams.
摘要：
背景：矿物质代谢对于骨骼和牙列的硬组织的正常发育至关重要。牙槽骨复合体包括以下4种矿化组织：牙釉质，牙本质,牙骨质,和牙槽骨。这些组织的发育过程受到遗传性疾病的影响，这些疾病会破坏磷酸盐和焦磷酸盐的稳态，尽管表现与骨骼中的不同。
方法：作者讨论了实验和比较分析的原始数据，并回顾了描述遗传性磷酸盐和焦磷酸盐疾病对牙齿组织影响的文章。特别强调这些疾病的新治疗方法如何影响受影响患者的口腔健康和牙科治疗。
结果：磷酸盐和焦磷酸盐代谢紊乱可导致软组织矿化减少（低矿化）或不适当（异位）钙化。X连锁低磷酸盐血症和高磷酸盐血症家族性肿瘤钙质沉着症中磷酸盐水平的破坏以及低磷酸盐血症中的焦磷酸盐水平的破坏和婴儿期的全身性动脉钙化会导致牙齿矿化缺陷。传统上,很少有选择来改善这些疾病引起的牙齿健康问题。新的抗体和酶替代疗法为改善受影响患者的口腔健康带来了可能性。
结论：过去20年的研究以指数方式扩展了对矿物质代谢的理解，并为矿化障碍带来了新的治疗方法。新实施和新兴的治疗策略会影响牙槽骨复合体，并与牙科治疗必须考虑的口腔保健方面相互作用。临床试验设计，和多学科护理团队的协调。
方法：作者讨论了实验和比较分析的原始数据，并回顾了描述遗传性磷酸盐和焦磷酸盐疾病对牙齿组织影响的文章。特别强调这些疾病的新治疗方法如何影响受影响患者的口腔健康和牙科治疗。
结果：磷酸盐和焦磷酸盐代谢紊乱可导致软组织矿化减少（低矿化）或不适当（异位）钙化。X连锁低磷酸盐血症和高磷酸盐血症家族性肿瘤钙质沉着症中磷酸盐水平的破坏以及低磷酸盐血症中的焦磷酸盐水平的破坏和婴儿期的全身性动脉钙化会导致牙齿矿化缺陷。传统上,很少有选择来改善这些疾病引起的牙齿健康问题。新的抗体和酶替代疗法为改善受影响患者的口腔健康带来了可能性。
结论：过去20年的研究以指数方式扩展了对矿物质代谢的理解，并为矿化障碍带来了新的治疗方法。新实施和新兴的治疗策略会影响牙槽骨复合体，并与牙科治疗必须考虑的口腔保健方面相互作用。临床试验设计，和多学科护理团队的协调。
