Dental Treatment Considerations in a Child with Pompe Disease -A Case Report

龐貝氏症兒童在牙科治療時的考量－病例報告

朱冠宇(Kuan-Yu Chu) ； 王姻麟(Yin-Lin Wang) ； 張曉華(Hsiao-Hua Chang) ； 黃桂芬(Guay-Fen Huang) ； 郭敏光(Ming-Kuang Guo)

《臺灣兒童牙醫學雜誌》 16卷4期 (2016/12) Pp. 153-158

https://doi.org/10.6319/TJPD.2016.16(4).4

摘要

Pompe disease is a rare autosomal recessive disorder results from the deficiency of acid α-glycosidase which leads to accumulation of glycogen in cells throughout the body. Patients with infantile onset Pompe disease may die from cardiorespiratory failure or respiratory infection in the first year of life. Enzyme replacement treatment (ERT) has prolonged the life span of patient with this disease. Therefore, as children with infantile Pompe disease live longer represent challenge to the pediatric dentist for the need of dental treatment. This case is a case of 5-year-9-month boy with infantile Pompe disease, was referred to our pediatric dental clinic for examination. He showed a long face with mouth opening posture, head lag, prognathic mandible. Intraoral examination revealed poor oral hygiene, multiple caries on primary posterior teeth, and anterior open-bite. He received composite resin restoration, pulp therapy and stainless steel crown restorations. Dental treatment for children with infantile Pompe disease is similar to those for normal children. However, attention must pay to the disease features during dental treatment procedures.

關鍵字

Pompe disease ； Dental treatment

並列摘要

龐貝氏症是一個極罕見的體染色體隱性遺傳疾病，因為缺乏一種負責分解肝醣的酵素（酸性α-葡萄糖甘脢），而使得肝醣會在全身的細胞中堆積。在以往比較嚴重的嬰兒型龐貝氏症患者常在出生後第一年就因為心臟衰竭或是呼吸系統感染而過世，幸好在酵素替代療法發展之後，已經能有效的控制龐貝氏症患者的病程及延長他們的壽命。因此在未來會有愈來愈多的嬰兒型龐貝氏症患童需要接受牙科治療，這對於兒童牙科醫師來說是一個新的挑戰。此案例報告是一位患有嬰兒型龐貝氏症的五歲男童，由小兒科醫師轉診進行牙齒檢查。他口外觀呈現長臉型及下顎突出；口內觀呈現維持不良的口腔清潔、多顆後牙齲齒及前牙開咬。因此為他進行齲齒的治療。在臨床上對於嬰兒型龐貝氏症患童的牙科治療與一般孩童沒有太大的差別，但是在牙科治療時仍然要留心於這項疾病的特色及可能會對治療造成的影響。

並列關鍵字

龐貝氏症 ； 牙科治療