Gomes Mônica Fernandes, Sichi Luigi Giovanni Bernardo, Giannasi Lilian Chrystiane, Amorim José Benedito Oliveira, da Rocha João Carlos, Koga-Ito Cristiane Yumi, Salgado Miguel Angel Castillo
Center of Biosciences Applied to Patients with Special Health Care Needs (CEPAPE), Institute of Science and Technology, São Jose dos Campos Campus, São Paulo State University-UNESP, São Paulo, SP, Brazil.
Dental School, Metropolitan University of Santos (UNIMES), Santos, SP, Brazil.
Case Rep Dent. 2018 Mar 20;2018:2409212. doi: 10.1155/2018/2409212. eCollection 2018.
Ectodermal dysplasia (ED) is a rare hereditary disorder affecting the development of ectoderm-derived organs and tissues. The aim of this study was to describe phenotypic features and the therapeutic approach in dentistry among three patients with ED, correlating their data with the literature. Additionally, to investigate the salivary gland disorders and their impacts on oral microbiota, we performed salivary tests, including salivary flow rate, salivary buffering capacity, and concentration levels of mutans streptococci, lactobacilli, and yeasts. All patients presented oligodontia, resulting in a significant masticatory dysfunction and aesthetic impairment. The counts of mutans streptococci (=3) and yeasts (=2) were high; on the other hand, the count of lactobacilli (=3) was low. Therefore, salivary and microbiological tests showed that the patients with ED, particularly the hypohidrotic type, presented a high risk of enamel caries and susceptibility to oral infections, which may be likely triggered by reduction of salivary flow and/or possible immunological disorders.
外胚层发育不全（ED）是一种罕见的遗传性疾病，影响外胚层衍生器官和组织的发育。本研究的目的是描述3例ED患者的表型特征和牙科治疗方法，并将他们的数据与文献进行关联。此外，为了研究唾液腺疾病及其对口腔微生物群的影响，我们进行了唾液检测，包括唾液流速、唾液缓冲能力以及变形链球菌、乳酸菌和酵母菌的浓度水平。所有患者均出现少牙症，导致明显的咀嚼功能障碍和美观受损。变形链球菌（=3）和酵母菌（=2）的计数较高；另一方面，乳酸菌（=3）的计数较低。因此，唾液和微生物检测表明，ED患者，尤其是无汗型患者，患釉质龋的风险较高，且易患口腔感染，这可能是由唾液分泌减少和/或可能的免疫紊乱引发的。
