Juloski Jovana, Dumančić Jelena, Šćepan Ivana, Lauc Tomislav, Milašin Jelena, Kaić Zvonimir, Dumić Miroslav, Babić Marko
Department of Orthodontics, School of Dental Medicine, University of Belgrade, Gastona Gravijea 2, 11000 Belgrade, Serbia.
Department of Dental Anthropology, School of Dental Medicine, University of Zagreb, Gundulićeva 5, 10000 Zagreb, Croatia; Department of Dental Medicine, University Hospital Center Zagreb, Gundulićeva 5, 10000 Zagreb, Croatia.
Arch Oral Biol. 2016 Nov;71:10-15. doi: 10.1016/j.archoralbio.2016.06.012. Epub 2016 Jun 16.
Turner syndrome occurs in phenotypic females with complete or partial absence of X chromosome. The leading symptom is short stature, while numerous but mild stigmata manifest in the craniofacial region. These patients are commonly treated with growth hormone to improve their final height. The aim of this study was to assess the influence of long-term growth hormone therapy on craniofacial morphology in Turner syndrome patients.
In this cross-sectional study cephalometric analysis was performed on 13 lateral cephalograms of patients with 45,X karyotype and the average age of 17.3 years, who have received growth hormone for at least two years. The control group consisted of 13 Turner syndrome patients naive to growth hormone treatment, matched to study group by age and karyotype. Sixteen linear and angular measurements were obtained from standard lateral cephalograms. Standard deviation scores were calculated in order to evaluate influence of growth hormone therapy on craniofacial components.
In Turner syndrome patients treated with growth hormone most of linear measurements were significantly larger compared to untreated patients. Growth hormone therapy mainly influenced posterior face height, mandibular ramus height, total mandibular length, anterior face height and maxillary length. While the increase in linear measurements was evident, angular measurements and facial height ratio did not show statistically significant difference. Acromegalic features were not found.
Long-term growth hormone therapy has positive influence on craniofacial development in Turner syndrome patients, with the greatest impact on posterior facial height and mandibular ramus. However, it could not compensate X chromosome deficiency and normalize craniofacial features.
特纳综合征发生于X染色体完全或部分缺失的表型女性。主要症状是身材矮小,而在颅面部区域有许多轻微的特征表现。这些患者通常接受生长激素治疗以提高其最终身高。本研究的目的是评估长期生长激素治疗对特纳综合征患者颅面部形态的影响。
在这项横断面研究中,对13例核型为45,X且平均年龄为17.3岁、接受生长激素治疗至少两年的患者的头颅侧位片进行了头影测量分析。对照组由13例未接受生长激素治疗的特纳综合征患者组成,根据年龄和核型与研究组匹配。从标准头颅侧位片中获得了16项线性和角度测量值。计算标准差分数以评估生长激素治疗对颅面部各组成部分的影响。
与未治疗的患者相比,接受生长激素治疗的特纳综合征患者的大多数线性测量值明显更大。生长激素治疗主要影响后脸高度、下颌升支高度、下颌总长度、前脸高度和上颌长度。虽然线性测量值的增加很明显,但角度测量值和面部高度比没有显示出统计学上的显著差异。未发现肢端肥大症特征。
长期生长激素治疗对特纳综合征患者的颅面部发育有积极影响,对后脸高度和下颌升支的影响最大。然而,它不能弥补X染色体缺陷并使颅面部特征正常化。