Johnson Euphemia LaRee, Roberts Michael W, Guckes Albert D, Bailey L'Tanya J, Phillips Ceib L, Wright J Timothy
Private Pediatric Dentistry Practice, Rocky Mount, North Carolina, USA.
Am J Med Genet. 2002 Nov 1;112(4):327-34. doi: 10.1002/ajmg.10654.
Ectodermal dysplasias (ED) are a heterogeneous group of inheritable disorders characterized by abnormal development of embryologic ectoderm derivatives. The purposes of this study were to: 1) create baseline cephalometric norms for male children with ED; 2) assess craniofacial growth and development in hypohidrotic ED male children with severe hypodontia, compared with non-ED children with class I dental relationships; 3) compare the craniofacial morphology of titanium dental implant-treated ED males with non-implant-treated ED males; and 4) correlate the severity of hypodontia to craniofacial dysmorphology. Cephalometric radiographs of class I individuals and implant-treated and nontreated ED groups were used to evaluate craniofacial morphology. Traditional cephalometric landmarks and measurements were used to compare groups using the generalized estimate equation analysis. Age, gender, and the number of permanent maxillary teeth present had a significant (P =.01) explanatory relationship with the craniofacial measures when comparing untreated ED children to norms. Mean craniofacial differences between ED and non-ED children still existed when the explanatory effects of these variables were controlled, indicating dysmorphology in several craniofacial structures (e.g., cranial base, mandibular length). The number of missing maxillary permanent teeth was significantly related with craniofacial dysmorphology in the ED population. Craniofacial morphology did not differ significantly between implant-treated and nontreated ED children, suggesting that treatment with intraosseous dental implants, as applied in this population, did not rescue normal craniofacial growth and development.
外胚层发育不全（ED）是一组遗传性疾病，其特征是胚胎外胚层衍生物发育异常。本研究的目的是：1）建立男性ED患儿的头影测量基线标准；2）与I类牙关系的非ED儿童相比，评估重度牙列缺损的少汗型ED男性儿童的颅面生长发育情况；3）比较钛牙种植体治疗的ED男性与未进行种植体治疗的ED男性的颅面形态；4）将牙列缺损的严重程度与颅面畸形相关联。使用I类个体以及种植体治疗组和未治疗组的ED患者的头影测量X线片来评估颅面形态。采用传统的头影测量标志点和测量方法，使用广义估计方程分析对各组进行比较。在将未治疗的ED儿童与标准进行比较时，年龄、性别和上颌恒牙的数量与颅面测量值具有显著（P = 0.01）的解释性关联。当控制这些变量的解释作用时，ED儿童与非ED儿童之间的平均颅面差异仍然存在，表明在几个颅面结构（如颅底、下颌长度）中存在畸形。上颌恒牙缺失的数量与ED人群的颅面畸形显著相关。种植体治疗的ED儿童与未治疗的ED儿童的颅面形态没有显著差异，这表明在该人群中应用骨内牙种植体治疗并不能挽救正常的颅面生长发育。
