乳児性ポンペ病の18ヶ月の子供。口腔徴候

An 18-Month-Old Child with Infantile Pompe Disease: Oral Signs.

抄録

ポンペ病の口腔内所見を文献に寄稿し，情報プラットフォームを構築することを目的としている．1歳半のPompe病患者（男性）が歯肉の腫脹のために当院に紹介された．初診時には，上顎右側前歯部に流動性のない正常な歯肉色の腫脹を認めた．両親はその部分を指でマッサージするように勧められた。半年後、51番、52番、62番、74番の歯が生え、51番と52番の歯は癒合し、84番の歯が生えているのが確認され、この歯の部位には前の歯と同様の腫れが見られました。この部位にも指でのマッサージを勧め、1週間後のフォローアップでは腫れが軽減していることが確認されました。歯の萌出の問題と発達期の歯の異常は、ポンペ病の徴候に含まれるべきである。

We aim to create an information platform by contributing orodental findings of Pompe disease to literature. An 18-month-old male patient with Pompe disease was referred to our clinic due to swelling of the gums. In first dental examination, a nonfluctuant, normal gingiva colored swelling at the right anterior region of maxilla was detected. His parents were recommended to perform finger massage to the region. Six months later, 51, 52, 62, and 74 numbered teeth had erupted, there was a fusion between 51 and 52 numbered teeth, 84 numbered tooth was seen to be erupted, and a swelling at the site of this tooth, similar to previous one, was present. Finger massage was recommended for this area as well, and the swelling was found to have decreased at the follow-up, one week later. Tooth eruption problems and developmental dental abnormalities should be included in the signs for Pompe disease.