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題 名 | Hereditary Gingival Fibromatosis and its Three Generations Pedigree--Case Report=遺傳性牙齦纖維腫大症--病例報告及三代家族圖譜 |
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作 者 | 項家蘭; 郭倍榮; 湯寶芳; | 書刊名 | 中華牙醫學雜誌 |
卷 期 | 18:1 1999.03[民88.03] |
頁 次 | 頁49-54 |
分類號 | 416.94 |
關鍵詞 | 遺傳性牙齦纖維腫大症; 三代家族譜; Hereditary gingival fibromatosis; Three-generation pedigree; |
語 文 | 英文(English) |
中文摘要 | 遺傳性牙齦纖維腫大症為一種罕見病例,通常為顯性遺傳但亦有隱性遺傳。臨床 上,上下顎牙齦有明顯的過度肥大現象,除牙齦觸摸時感覺較正常牙齦堅實外,其顏色與正 常牙齦無異。此病症通常單獨發生,偶而亦伴隨其它症候群一同發生,最常被報導的是多毛 症、癲癇及智障。本病例為六歲九個月之男孩,由於牙齦腫大,蓋至牙冠部份,口腔清潔不 易維持,導致齲齒形成,故來求診。經口腔檢查,除牙齦腫大外,並有多顆鄰接面齲齒,包 括上下顎雙側第一、第二乳臼齒,以及上顎正中乳門齒。本病例並未伴隨其他症候群。組織 切片顯現增生緻密纖維結締組織,其上覆蓋具有釘小網之多層鱗狀上皮以及黏膜下層有輕微 發炎現象。由於其血親中如祖父、父親、叔父、兄長、堂妹亦有相同之情形,故符合自體顯 性遺傳的特性。綜合其病史及家族遺傳特性診斷為遺傳性牙齦纖維腫大症。因此先替病患進 行各項修復治療,再建議病患定期追蹤檢查及接受牙齦切除術處理。 |
英文摘要 | Hereditary gingival fibromatosis is a rare disorder and usually inherits as an autosomal dominant condition, though recessive form has been reported. It can occur as an isolated entity or as part of a syndrome, such as hirsutism, epilepsy and mental retardation. Clinically, the gingivae of the maxilla and mandible are hyperplastic, but the tissues are firm to the touch without other abnormal color alteration. A case of isolated gingival fibromatosis affecting five members among three generations was reported. Clinical findings and pedigree were discussed. Histopathologic examination revealed hyperplastic dense fibrous connective tissue covered by stratified epithelium with elongated rete ridges and mild inflammation in the submucosa. A diagnosis of hereditary gingival fibromatosis was made from the clinical features, family history and histopathologic findings. The patient was placed on a periodic recall schedule to evaluate the appropriate timing of gingivectomy after all restorative treatments had been performed. |
本系統中英文摘要資訊取自各篇刊載內容。