PDF(Pubmed)
Abstract：
Background and Objectives: Gaucher disease (GD) is a lysosomal storage disorder with the genetic autosomal recessive transmission. Bone involvement is a prevalent finding in Gaucher disease. It causes deformity and limits daily activities and the quality of life. In 75% of patients, there is bone involvement. This review aims to evaluate the principal findings in the jaw by a Cone-beam computed tomography (CBTC) and X-ray orthopantomography; Materials and Methods: PubMed, Web of Science, Lilacs and Scopus were systematically searched until 31 December 2022. In addition, a manual search was performed using the bibliography of selected articles and a Google Scholar search. Clinical studies were selected that considered principal radiographic findings in radiography in a group of patients affected by GD. Results: Out of 5079 papers, four studies were included. The main findings are generalized rarefaction and enlarged narrow space, anodontia. Conclusions: The exact mechanism of bone manifestation is probably due to the infiltration of Gaucher cells in the bone marrow and, consequently, the destruction of bone architecture. All long bones are a potential means of skeletal manifestation. The jaw is more affected than the maxilla, and the principal features are cortical thinning, osteosclerosis, pseudocystic lesions, mental demineralization, flattening in the head of the condyle, effacement of anatomical structures, thickening of maxillary sinus mucosa. The dentist plays a crucial role in diagnosing and treating these patients. Sometimes the diagnosis can be made by a simple panoramic radiograph. All long bones are affected, and the mandible is particularly involved.
摘要：
背景与目的:戈谢病(GD)是一种常染色体隐性遗传传递的溶酶体贮积症。骨受累是戈谢病的普遍发现。它会导致畸形并限制日常活动和生活质量。在75%的患者中，有骨头受累。这篇综述旨在通过锥形束计算机断层扫描（CBTC）和X射线矫形器造影术评估颌骨的主要发现；材料和方法：PubMed，WebofScience,对丁香花和Scopus进行了系统搜索，直到2022年12月31日。此外,使用选定文章的参考书目和Google学者搜索进行了手动搜索。选择的临床研究考虑了一组受GD影响的患者的X线摄影中的主要X线摄影结果。结果：在5079篇论文中，纳入四项研究。主要发现是广义稀疏和扩大的狭窄空间，牙齿缺失症.结论:骨表现的确切机制可能是由于骨髓中Gaucher细胞的浸润,因此,骨骼结构的破坏。所有长骨都是骨骼表现的潜在手段。颌骨比上颌骨受影响更大,主要特征是皮质变薄，骨硬化,假性囊肿病变,精神脱矿，在髁的头部变平，解剖结构的消失，上颌窦粘膜增厚。牙医在诊断和治疗这些患者中起着至关重要的作用。有时可以通过简单的全景X光片进行诊断。所有的长骨都受到影响,尤其是下颌骨。
