McGovern E, Fleming P, Costigan C, Dominguez M, Coleman D C, Nunn J
Dental Dept., Our Lady's Children's Hospital, Crumlin, Dublin 12, Ireland.
Eur Arch Paediatr Dent. 2008 Dec;9(4):236-44. doi: 10.1007/BF03262642.
APECED (Autoimmune Polyendocrinopathy Candidiasis Ectodermal Dystrophy) is a rare autosomal recessive disease characterised primarily by sequential immune-mediated destruction of endocrine tissues, chronic oral or mucocutaneous candidiasis and ectodermal disorders, including hypoplasia of dental enamel.
This was to investigate the oral health and presence of enamel defects in a cohort of patients with APECED.
16 patients with APECED (mean age of 13.9 years) were matched for age and gender with healthy controls. A comprehensive medical, dental and drug history was recorded, followed by a clinical assessment of oral health which was determined by assessing periodontal treatment needs, prevalence of dental caries, erosion, fluorosis and enamel defects. The estimated time of the development of the enamel defects and the contemporaneous medical diagnosis were recorded.
Oral health of patients with APECED was poor compared with controls, with a higher prevalence of periodontal disease, caries and erosion. There was a significantly (P < 0.05) higher prevalence of enamel defects in the study group. The enamel defects were mostly hypoplastic in the form of pits, missing enamel and grooves. The enamel defects occurred in a chronological pattern. There was a strong association between the estimated time of defective enamel formation and a history of hypoparathyroidism. Gastrointestinal dysfunction and a history of chronic mucocutaneous candidiasis were also associated with the presence of enamel defects.
The oral health of individuals with APECED was poor compared with controls with a higher prevalence of periodontal disease, caries, erosion and enamel defects. The enamel defects in the study population occurred in a chronological pattern and some were associated with a history of systemic disease during the period of tooth development.
自身免疫性多内分泌腺病-念珠菌病-外胚层营养不良（APECED）是一种罕见的常染色体隐性疾病，主要特征为内分泌组织的免疫介导性序贯破坏、慢性口腔或黏膜皮肤念珠菌病以及外胚层疾病，包括牙釉质发育不全。
本研究旨在调查一组APECED患者的口腔健康状况及牙釉质缺陷情况。
16例APECED患者（平均年龄13.9岁）按年龄和性别与健康对照进行匹配。记录全面的医学、牙科和用药史，随后进行口腔健康临床评估，通过评估牙周治疗需求、龋齿、侵蚀、氟斑牙和牙釉质缺陷的患病率来确定。记录牙釉质缺陷的估计发生时间及同期的医学诊断。
与对照组相比，APECED患者的口腔健康状况较差，牙周病、龋齿和侵蚀的患病率更高。研究组牙釉质缺陷的患病率显著更高（P<0.05）。牙釉质缺陷大多为发育不全，表现为凹坑、牙釉质缺失和沟纹。牙釉质缺陷按时间顺序出现。牙釉质形成缺陷的估计时间与甲状旁腺功能减退病史之间存在密切关联。胃肠功能障碍和慢性黏膜皮肤念珠菌病史也与牙釉质缺陷的存在有关。
与对照组相比，APECED患者的口腔健康状况较差，牙周病、龋齿、侵蚀和牙釉质缺陷的患病率更高。研究人群中的牙釉质缺陷按时间顺序出现，部分与牙齿发育期间的全身性疾病史有关。
