β-型地中海貧血之口腔顏面部徵狀及牙科治療－病例報告

Beta-Thalassemia Major and Considerations for Dental Treatment: A Case Report

王馨慧(Hsin-Hui Wang) ； 王姻麟(Yin-Lin Wang) ； 張曉華(Hsio-Hua Chang) ； 黃桂芬(Guay-Fen Huang) ； 郭敏光(Ming-Kuang Guo)

《臺灣兒童牙醫學雜誌》 14卷3期 (2014/09) Pp. 90-94

https://doi.org/10.6319/TJPD.2014.14(3).3

摘要

地中海貧血乃是遺傳性的溶血性貧血疾病，其中最為嚴重的類型就是β-型地中海貧血。此篇病歷報告是描述一位兩歲十一個月β-型地中海貧血的女童，具有典型的身體系統性特徵，但並不具明顯的典型口腔顏面部特徵，包含花栗鼠臉型、嚴重上顎骨突出、上唇回縮、上前牙外凸、齒間縫隙大、嚴重程度不等的咬合不正情形。在數次約診以適當的行為引導下，蛀牙均接受根管治療及不鏽鋼牙冠復形，嚴重齲齒的上顎前牙接受拔牙並製作活動假牙。每次約診的安排和診療的進行都安排在病患定期輸血後。術前術後建議家長要注重女童之口腔衛生的實施、飲食習慣諮詢和定期口腔檢查。經過一年追蹤，經過治療及予以保留的乳齒均無明顯徵狀，口腔衛生改善，活動假牙也功能良好。β-型地中海貧血病童的牙科治療需要仔細術前評估及建立良好的口腔衛生照護。

關鍵字

β-型地中海貧血 ； 口腔顏面徵狀 ； 牙科治療

並列摘要

Thalassemias are hereditary hemolytic anemia diseases, among which β-thalassemia major is the most severe type. This case is a 2-year-11-month old girl with β-thalassemia major who presented typical systemic feature, but without significant typical craniofacial features including chipmunk face, severe maxillary protrusion, upper lip retraction, anterior teeth protrusion, interdental spaces, large and varied degrees of malocclusion. With proper behavior management, carious teeth had received pulpal therapy and stainless steel crown restorations. Severe carious upper anterior teeth were extracted and followed with removable denture replacement. The dental treatments were scheduled and proceeded right after her regular blood transfusions. Oral hygiene instruction and practice, dietary consultation and periodic examination have been emphasized and advised. After 1 year's follow-up, all remaining teeth remained free from signs and symptoms. The removable denture functioned well. β-thalassemia major patients need prudential planning before proceeding dental treatment.

並列關鍵字

Beta-thalassemia major ； Orofacial manifestations ； Dental treatmentt