Abstract：
Diamond Blackfan anemia (DBA) is a chronic congenital form of erythrocytic hypoplasia in which erythroid precursor cell levels are low. DBA reflects ribosomal dysfunction and is accompanied by hematopoietic cell apoptosis, anemia, and various somatic symptoms. We report the characteristic symptoms of the craniofacial region and the orthodontic treatments of two DBA cases. Case 1 was a 12-year-old female. The typical physical and facial characteristics of DBA were lacking. On initial examination, she exhibited a skeletal Class II jaw and end to end molar relationships and a large overjet. An edgewise appliance was placed after extraction of the first maxillary premolars. After 3 years and 11 months, an appropriate overjet and overbite, rigid intercuspation, and an acceptable profile were evident without any clinical adverse effects. Case 2 was a 13-year-old female. She exhibited a skeletal Class I jaw relationship, a spaced dental arch, the maxillofacial dysplasia characteristic of Binder syndrome, hypoplasia of the right mandibular condyle, and labial protrusions of the maxillary and mandibular incisors. We placed an edgewise appliance and after 1 year and 7 months, the occlusion was optimal in the absence of any adverse effects. Our two DBA cases exhibited a broad spectrum of physical and dentofacial symptoms. Patients with DBA are often prescribed combined steroid/bisphosphonate therapies. Both agents are likely to affect alveolar bone remodeling after tooth extraction and orthodontic tooth movement. Careful consideration of medication with reference to various dentofacial characteristics is necessary.
摘要：
DiamondBlackfan贫血（DBA）是一种慢性先天性红细胞发育不全，其中红系前体细胞水平较低。DBA反映了核糖体功能障碍，并伴有造血细胞凋亡，贫血,和各种躯体症状。我们报告了两个DBA病例的颅面区域的特征性症状和正畸治疗。病例1为12岁女性。DBA缺乏典型的身体和面部特征。在初步检查中，她表现出骨骼的II级下颌和端到端磨牙关系以及大的过度喷射。拔除第一上颌前磨牙后放置边缘矫治器。3年零11个月后,适当的过喷和过咬，刚性咬合，和可接受的概况是明显的，没有任何临床不良反应。病例2是一名13岁的女性。她表现出骨骼一级的下巴关系，一个间隔开的牙弓，Binder综合征的颌面部发育不良特征，右下颌髁突发育不全,上颌和下颌切牙的唇突起。我们放置了一个边缘设备，在1年零7个月后，在没有任何不良反应的情况下,封堵效果最佳.我们的两个DBA病例表现出广泛的身体和牙面部症状。DBA患者通常被处方联合类固醇/双膦酸盐治疗。两种药物都可能影响拔牙和正畸牙齿移动后的牙槽骨重塑。必须根据各种牙面特征仔细考虑药物。
