Davidovich Esti, Eimerl Dan, Aker Memet, Shapira Joseph, Peretz Benjamin
Department of Pediatric Dentistry, The Hebrew University-Hadassah School of Dental Medicine, Jerusalem, Israel.
Pediatr Dent. 2005 May-Jun;27(3):244-8.
Dyskeratosis congenita (DKC) is a rare syndrome characterized by cutaneous hyperpigmentation, nail dystrophy, leukoplakia, and pancytopenia. The purpose of this case study was to describe the management of a 7-year-old girl diagnosed with DKC who urgently needed dental treatment under general anesthesia before bone marrow transplantation (BMT). The patient presented normal skin, nails, and hair, but oral examination revealed a number of ulcers, leukoplakia, gingival recessions, alveolar bone loss, and dental caries. Hematologic preparation included raising blood parameters, and the anesthesiologist to had consider pulmonary infection. The alveolar bone loss and the gingival recessions required the consultation of a periodontist. Avoiding stainless steel crowns was necessary due to potential plaque accumulation in the crown margins. The goal of this dental treatment was eliminating potential sources of infection before transplantation was conducted. It is important for the pediatric dentist to recognize the medical aspects associated with dental management prior to BMT, and to incorporate them into the treatment plan.
先天性角化不良（DKC）是一种罕见的综合征，其特征为皮肤色素沉着、指甲营养不良、黏膜白斑和全血细胞减少。本病例研究的目的是描述一名7岁诊断为DKC的女孩在骨髓移植（BMT）前急需在全身麻醉下进行牙科治疗的处理情况。该患者皮肤、指甲和头发正常，但口腔检查发现多处溃疡、黏膜白斑、牙龈退缩、牙槽骨丧失和龋齿。血液学准备包括提高血液参数，麻醉医生需要考虑肺部感染。牙槽骨丧失和牙龈退缩需要牙周病医生会诊。由于牙冠边缘可能积聚菌斑，避免使用不锈钢牙冠是必要的。此次牙科治疗的目标是在进行移植前消除潜在的感染源。儿科牙医认识到BMT前与牙科治疗相关的医学问题并将其纳入治疗计划非常重要。
