Universidade Federal de Minas Gerais, Faculdade de Odontologia, Departamento de Saúde Bucal da Criança e do Adolescente, Belo Horizonte, Brasil.
Universidade Federal de Minas Gerais, Faculdade de Odontologia, Departamento de Clínica, Patologia e Cirurgia, Belo Horizonte, Brasil.
J Appl Oral Sci. 2021 Apr 14;29:e20200978. doi: 10.1590/1678-7757-2020-0978. eCollection 2021.
Mucopolysaccharidosis (MPS) is a group of rare and inherited metabolic disorders caused by the accumulation of macromolecule glycosaminoglycans inside lysosomes. Affected individuals may have dental and craniofacial tissue alterations, facilitating the development of several oral diseases.
To assess, with panoramic radiographic images, the frequency of dental and maxillomandibular incidental findings among MPS individuals and compare them with non-MPS individuals.
A cross-sectional study evaluating a sample of 14 MPS individuals and 28 non-MPS individuals aged from 5 to 26 years was carried out. They were matched for sex and age on a 2:1 proportion. Panoramic radiographs were assessed for the presence/absence of the following dental and maxillomandibular alterations: dental anomalies of number (hypodontia/dental agenesis, supernumerary teeth); anomalies of form (microdontia, macrodontia, conoid teeth, taurodontism, and root dilaceration); anomalies of position (impacted tooth, inverted tooth, tooth migration, partially bony teeth, complete bony teeth); periapical alterations (furcation lesion, circumscribed bone rarefaction); other alterations (radiolucent bone lesions, radiopaque bone lesions, radiopacity in the maxillary sinus, condylar hypoplasia). Differences between groups were tested by the Fisher's exact test and chi-square test (p<0.05).
For intrarater agreement, Kappa values were 0.76 to 0.85. The presence of supernumerary teeth (p=0.003); conoid teeth (p=0.009); taurodontism (p<0.001); impacted teeth (p<0.001); partial bony teeth (p=0.040); complete bony teeth (p=0.013); and root dilaceration (p=0.047) were statistically more frequent in MPS individuals compared to non-MPS individuals. Bone rarefaction/furcation lesions (p=0.032), condylar hypoplasia (p<0.001), radiolucent bone lesions (p=0.001), and dentigerous cysts (p=0.002) were also more frequent in MPS individuals.
The presence of specific oral manifestations is more common in MPS individuals than non-MPS individuals.
黏多糖贮积症（MPS）是一组由溶酶体内大分子糖胺聚糖积累引起的罕见遗传性代谢疾病。受影响的个体可能有牙和颅面组织改变，从而促进了几种口腔疾病的发展。
通过全景影像学评估 MPS 个体的牙齿和颌面部偶然发现的频率，并与非 MPS 个体进行比较。
进行了一项横断面研究，评估了 14 名 MPS 个体和 28 名年龄在 5 至 26 岁之间的非 MPS 个体的样本。按照 2:1 的比例按性别和年龄进行匹配。评估全景片是否存在/不存在以下牙齿和颌面部改变：牙齿数量异常（缺牙/牙缺失，多生牙）；形态异常（小牙，巨牙，锥形牙，尖牙型，牙根扭曲）；位置异常（阻生牙，倒置牙，牙迁移，部分骨性牙，完全骨性牙）；根尖周改变（分叉病变，局限性骨稀疏）；其他改变（透光性骨病变，不透光性骨病变，上颌窦内不透光性，髁突发育不良）。Fisher 确切检验和卡方检验（p<0.05）用于检验组间差异。
对于内部观察者的一致性，Kappa 值为 0.76 至 0.85。多生牙（p=0.003）；锥形牙（p=0.009）；尖牙型（p<0.001）；阻生牙（p<0.001）；部分骨性牙（p=0.040）；完全骨性牙（p=0.013）；和牙根扭曲（p=0.047）在 MPS 个体中比非 MPS 个体更常见。骨稀疏/分叉病变（p=0.032），髁突发育不良（p<0.001），透光性骨病变（p=0.001）和含牙囊肿（p=0.002）在 MPS 个体中也更为常见。
与非 MPS 个体相比，MPS 个体中存在特定的口腔表现更为常见。
