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重型地中海贫血患者的颅面特征。

Craniofacial Characteristics of Thalassemia Major Patients.

作者信息

Karakas Sacide, Tellioglu Ayfer Metin, Bilgin Mehmet, Omurlu Imran Kurt, Caliskan Sercin, Coskun Salih

机构信息

Department of Anatomy, Adnan Menderes University School of Medicine, Aydın, Turkey.

Department of Biophysics, Adnan Menderes University School of Medicine, Aydın, Turkey.

出版信息

Eurasian J Med. 2016 Oct;48(3):204-208. doi: 10.5152/eurasianjmed.2016.150013.

Abstract

OBJECTIVE

Thalassemias major are the most common autosomal recessive disorders; they are characterized by anomalies in the synthesis of the beta chains of hemoglobin and are often associated with varying degrees of craniofacial anomalies. The purpose of this study was to evaluate the craniofacial dimensions of β-thalassemia patients and to identify differences by comparing them to those of a control group.

MATERIALS AND METHODS

The study comprised 43 thalassemia major patients and 26 age- and sex- matched healthy control subjects. Anthropometric measurements were performed in six different craniofacial regions (head, face, nose, mouth, eyes, and ears); a total of 23 craniofacial variables were measured.

RESULTS

Craniofacial measurements in the regions of the face, nose, lips and mouth, and ears in the thalassemia major patient group yielded statistically significant differences compared to those in the control group (p<0.05). However, no statistically significant differences were observed in the measurements of the head and eye regions.

CONCLUSION

The study increased our understanding of the craniofacial anatomy of thalassemia major patients and enabled us to obtain quantitative results.

摘要

目的

重型地中海贫血是最常见的常染色体隐性疾病;其特征是血红蛋白β链合成异常,常伴有不同程度的颅面畸形。本研究的目的是评估β地中海贫血患者的颅面尺寸,并通过与对照组比较来确定差异。

材料与方法

本研究包括43例重型地中海贫血患者和26例年龄及性别匹配的健康对照者。在六个不同的颅面区域(头部、面部、鼻子、嘴巴、眼睛和耳朵)进行人体测量;共测量了23个颅面变量。

结果

与对照组相比,重型地中海贫血患者组在面部、鼻子、嘴唇和嘴巴以及耳朵区域的颅面测量结果存在统计学显著差异(p<0.05)。然而,在头部和眼睛区域的测量中未观察到统计学显著差异。

结论

本研究增进了我们对重型地中海贫血患者颅面解剖结构的理解,并使我们能够获得定量结果。

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