戈谢病的骨骼受累情况：骨病程度、脾脏体积与生活质量
Skeletal involvement in Gaucher disease: extent of bone disease, splenic volume, and quality of life.
作者信息
de Mello Ricardo Andrade Fernandes, Mello Melissa Bosi Nonato, Pessanha Laís Bastos, Fonseca Ana Paula Alves
机构信息
Department of Internal Medicine, Universidade Federal do Espírito Santo (UFES), Vitória, ES, Brazil.
Centro Capixaba de Oncologia (Cecon), Vitória, ES, Brazil.
出版信息
Radiol Bras. 2021 Mar-Apr;54(2):71-76. doi: 10.1590/0100-3984.2020.0014.
OBJECTIVE
To investigate the correlations among the extent of bone involvement, splenic volume, and quality of life in patients with Gaucher disease.
MATERIALS AND METHODS
This was a descriptive, prospective cross-sectional study of 18 patients with Gaucher disease who underwent 3-T magnetic resonance imaging of both femurs and the lumbar spine. Semiquantitative analyses were performed on the basis of the bone marrow burden (BMB) score. We looked for linear relationships among the variables splenic volume, quality of life score, and BMB score.
RESULTS
We identified a linear relationship between the BMB scores and splenic volume. The quality of life score showed no statistically significant relationship with splenic volume or the BMB score.
CONCLUSION
The linear relationship between the BMB score and the splenic volume indicates that the extent of bone disease is greater in individuals with splenomegaly. No correlation was found between the BMB and quality of life scores, illustrating the insidious and silent progression of Gaucher disease.
目的
探讨戈谢病患者骨受累程度、脾脏体积与生活质量之间的相关性。
材料与方法
这是一项描述性、前瞻性横断面研究，对18例戈谢病患者进行了双侧股骨和腰椎的3T磁共振成像检查。基于骨髓负荷（BMB）评分进行半定量分析。我们研究了脾脏体积、生活质量评分和BMB评分之间的线性关系。
结果
我们发现BMB评分与脾脏体积之间存在线性关系。生活质量评分与脾脏体积或BMB评分之间无统计学显著关系。
结论
BMB评分与脾脏体积之间的线性关系表明，脾肿大患者的骨病程度更严重。未发现BMB评分与生活质量评分之间存在相关性，这说明了戈谢病隐匿且无症状的进展过程。
Zotero 插件