降钙素治疗第二牙过渡期进行性小天使症

Pub Date : 2023-11-09 DOI:10.1155/2023/2347855
Ethan Meijer, Henk van den Berg, Arjen H. G. Cleven, Esther Edelenbos, Willem H. Schreuder
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引用次数: 0

摘要

小天使病是一种常染色体显性遗传病。未经治疗的严重小天使病可能导致颌面骨骼严重畸形,发育牙齿细菌和牙齿。在疾病的活跃期,几乎没有描述和经验应用的介入治疗试图限制由扩大的骨内病变进展引起的损伤和变形。最终目标是一旦进行性生长停止,疾病进入静止阶段,将矫正手术的需要降到最低。对小天使病病理生理学的新见解假设在疾病的(超)激活中牙齿发育和颌骨生长的潜在作用。理论上,这可以引导药物干预的理想时刻。在本病例报告中,描述了全身性降钙素治疗的标签外使用,特别强调了其适当的治疗时间和持续时间的潜在重要性。
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Treatment of Progressive Cherubism during the Second Dental Transitional Phase with Calcitonin
Cherubism is an autosomal dominant disease with variable expression. Aggressive forms of untreated cherubism may lead to severe malformation of the maxillofacial skeleton, developing tooth germs and teeth. Scarcely described and empirically applied interventional therapies during active stages of the disease try to limit the damage and deformation caused by progression of expanding intraosseous lesions. The final goal is to minimize the need for corrective surgeries once progressive growth has halted and disease enters its quiescent phase. New insights into the pathophysiology of cherubism hypothesize a potential role for dental development and jaw growth in the (hyper)activation of the disease. Theoretically, this could guide the ideal moment of pharmacological interventions. In this case report, the off-label use of systemic calcitonin treatment is described, stressing particularly the potential importance of its appropriate timing and duration of treatment.
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