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Orthodontic treatment considerations for a patient with erythropoietic protoporphyria

机译:红细胞生成性原卟啉症患者的正畸治疗注意事项

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Erythropoietic protoporphyria (EPP) is an inherited blood disorder in which formation of the heme group of hemoglobin is defective. Specifically, a deficiency of the enzyme ferrochelatase leads to the accumulation of protoporphyrin, resulting in often painful photosensitivity of the skin and tissues. The prevalence of EPP is estimated at 1:75,000 to 1:200,000. Photosensitivity is exhibited upon exposure to light with specific wavelengths through the creation of reactive oxygen products (oxidants), activation of the complement system, and mast cell degranulation. The aim of this article is to report the orthodontic treatment of an 11-year-old boy with EPP, a Class III skeletal relationship, and an anterior crossbite. Orthodontic treatment established normal overbite and overjet. Short-term periodontal and dental tissue responses to treatment were noted. Extra care was needed when collecting photographic and radiographic records for this patient and during some treatment procedures to avoid causing a photosensitive reaction of the skin or oral mucosa.
机译:促红细胞生成原卟啉症(EPP)是一种遗传性血液疾病,其中血红蛋白血红素基团的形成存在缺陷。具体而言,缺乏铁螯合酶的酶会导致原卟啉积聚,导致皮肤和组织的光敏性常常令人痛苦。 EPP的患病率估计为1:75,000至1:200,000。通过产生活性氧产物(氧化剂),激活补体系统和肥大细胞脱粒,在暴露于特定波长的光时会表现出光敏性。本文的目的是报告对一名11岁的EPP,III类骨骼关系和前牙咬伤的男孩进行的正畸治疗。正畸治疗建立了正常的咬合和溢流。注意到短期牙周和牙齿组织对治疗的反应。在为该患者收集照片和射线照相记录时以及在某些治疗程序期间,需要格外小心,以免引起皮肤或口腔粘膜的光敏反应。

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