Department of Orthodontics, Center for Dental and Oral Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
Department of Pediatrics, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
Int J Paediatr Dent. 2022 Sep;32(5):737-744. doi: 10.1111/ipd.12952. Epub 2022 Apr 2.
Mucopolysaccharidoses (MPS) are a group of rare metabolic diseases characterized by a wide spectrum of symptoms including progressive condylar resorption.
The aim of this study was to quantify the severity of condylar involvement in MPS I individuals in comparison with a group of non-MPS individuals and to describe how condylar changes may vary among the different types of MPS.
Fifty panoramic radiographs of MPS patients (13.4 ± 6.2 years) with MPS I (n = 14), MPS II (n = 2), MPS IV (n = 8) and MPS VI (n = 2) were compared with forty panoramic radiographs of non-MPS individuals. The severity of condylar resorption was evaluated using a qualitative score (grades 0-3) and using the ratio of condylar height to ramus height (CH: RH).
All MPS I and VI individuals showed pronounced bilateral degenerative condylar resorption. In contrast, individuals with MPS II and IV exhibited heterogeneous findings. The quantification of condylar height to ramus height revealed that CH: RH was significantly decreased in MPS I as compared to that of non-MPS individuals (P < .001). In contrast, the CH: RH ratios of MPS II and IV showed great variability.
Mucopolysaccharidoses subtypes differ with regard to the severity of condylar resorption.
黏多糖贮积症（MPS）是一组罕见的代谢疾病，其特征为广泛的症状谱，包括进行性髁突吸收。
本研究旨在比较 MPS I 个体与非 MPS 个体之间髁突受累的严重程度，并描述不同类型 MPS 中髁突变化的差异。
50 例 MPS 患者（MPS I：14 例，MPS II：2 例，MPS IV：8 例，MPS VI：2 例）和 40 例非 MPS 患者的全景片进行比较。采用定性评分（0-3 级）和髁突高度与升支高度比（CH:RH）来评估髁突吸收的严重程度。
所有 MPS I 和 VI 个体均表现出明显的双侧退行性髁突吸收。相比之下，MPS II 和 IV 个体表现出异质性发现。CH:RH 的定量分析表明，MPS I 组的 CH:RH 明显低于非 MPS 个体（P<.001）。相比之下，MPS II 和 IV 组的 CH:RH 比值差异较大。
黏多糖贮积症各亚型的髁突吸收严重程度不同。
