- 期刊
Dental Management of Children with Ehlers-Danlos syndrome-Report of Two
鬆皮症患者之牙科照護－附二病歷報告
摘要
俗稱鬆皮症的愛何妲奴症候群(Ehlers-Danlos syndrome)是一種先天膠原結締組織異常的疾病，會造成患者體內合成膠原出現障礙，以致膠原不足或品質不好，其特徵包括皮膚有高度伸展性，皮膚和血管較為脆弱，傷口癒合比較慢，關節活動範圍的過度增加，故此疾病同時也被稱為橡皮人症候群(rubber man syndrome)。除了上述提及之變異之外，此類患者的心臟也可能會有二尖瓣膜脫垂，另外要注意因脊柱側凸而造成呼吸受限的情形。而此種病症目前在醫學上並沒有特殊的治療方法。此類患者在牙科的情況上，會有早發性牙周病的情形，甚至導致恆牙的早期脫落。另外在牙科的治療前需先確定患者之凝血功能情況，盡量避免會導致出血的治療，或在治療前後控制好病患之凝血功能。本篇報告將提出台北榮總兒童牙科治療一對患有鬆皮症兄妹的情形，並討論如何從身體，口腔照顧方面訂定整體的治療計畫。
並列摘要
Ehlers-Danlos syndrome (EDS) is a heterogeneous group of inheritable connective tissue disorders. There are collagen defects caused by gene mutation. No uniform guideline can be used to treat these patients due to multi-organ involvement and varied presentations of this disease, such as poor wound healing ability, hypermobile joint. However, there is no specific treatment for these patients.These patients usually present early-onset periodontitis leading to premature loss of permanent dentition. The bleeding tendency needs to be monitored before and after dental treatment. Dentists need to minimize bleeding during procedure. We report two cases with Ehlers-Danlos syndrome who presented extensive dental caries, and mental retardation. They received comprehensive dental treatment under general anesthesia to minimize the trauma from their uncooperative behavior.