Alves Patricia Valéria Milanezi, Alves Daniele Karina M, de Souza Margareth Maria Gomes, Torres Sandra Regina
Federal University of Rio de Janeiro, Orthodontics, Rio de Janeiro 21941-590, Brazil.
Angle Orthod. 2006 Mar;76(2):269-73. doi: 10.1043/0003-3219(2006)076[0269:OTOPWS]2.0.CO;2.
Sickle-cell anemia is a genetic blood disease characterized by a hemoglobin gene mutation. The genetic failure is basically constituted by replacement of the hemoglobin beta chain in the sixth position so that the amino acid valine is encoded instead of glutamic acid. As a result, the erythrocytes have their normal biconcave discoid shape distorted, generally presenting a sicklelike shape, which reduces both their plasticity and lifetime. Because a complete blood supply is so important during application of both intraoral and extraoral forces, this article addresses the general and oral aspects associated with sickle-cell anemia. This will guide the clinician regarding such patients who seek orthodontic treatment by making references to literature on multidisciplinary management.
镰状细胞贫血是一种由血红蛋白基因突变引起的遗传性血液疾病。这种基因缺陷主要是由于血红蛋白β链第六位的氨基酸被替换，导致编码的是缬氨酸而非谷氨酸。结果，红细胞正常的双凹圆盘状形态发生扭曲，通常呈现镰刀状，这降低了它们的可塑性和寿命。由于在施加口内和口外力时充足的血液供应非常重要，本文探讨了与镰状细胞贫血相关的一般情况和口腔方面的问题。这将通过参考多学科管理的文献，为寻求正畸治疗的此类患者的临床医生提供指导。
