戈谢病患者肝脾病变定量磁共振影像研究  

Quantitative Imaging Study of Liver and Spleen Lesions in Patients with Gaucher Disease

作　　者：李笛 陶晓娟[1] 张宁宁[1] 霍爱华[1] 胡迪[1] 吕艳秋[1] 张永红[2] 彭芸[1] LI Di;TAO Xiaojuan;ZHANG Ningning;HUO Aihua;HU Di;LYU Yanqiu;ZHANG Yonghong;PENG Yun(Department of Radiology,National Center for Children's Health,Beijing Children's Hospital,Capital Medical University,Beijing 100045,China;Department of Hematology,National Center for Children's Health,Beijing Children's Hospital,Capital Medical University,Beijing 100045,China)

机构地区：[1]国家儿童医学、中心首都医科大学附属北京儿童医院放射科,北京100045 [2]国家儿童医学、中心首都医科大学附属北京儿童医院血液科,北京100045

出　　处：《罕见病研究》2022年第3期283-288,共6页Journal of Rare Diseases

摘　　要：目的通过对治疗后戈谢病患者的肝脾系统病变进行定量影像学评估,以加深对戈谢病的了解。方法自1999年8月至2018年8月对来首都医科大学附属北京儿童医院就诊的戈谢病患儿进行登记和临床检查治疗,并对2018年8月来本院集中随诊的40例戈谢病患儿进行定量影像学研究,同时匹配了34名正常志愿者。所有受试者行磁共振成像扫描。并对受试者肝脾的脂肪分数(FF)、铁含量(R2^(∗))、标准表观扩散系数(sADC)、慢速表观扩散系数(D)、快速表观扩散系数(D^(∗))和灌注分数(f)等参数进行测量。对戈谢病患者和正常者所测得的定量参数值采用独立样本t检验。结果戈谢病患者的肝脾的脂肪分数(FF)、铁含量(R2^(∗))、标准表观扩散系数(sADC)、慢速表观扩散系数(D)、快速表观扩散系数(D^(∗))和灌注分数(f)等参数无显著性差异,肝脏弹性值也在正常范围内,但患者的肝脾体积与正常者呈明显差异。结论治疗后戈谢病患者除肝脾体积仍大于正常人,其余定量参数在正常范围内,说明长期的酶替代治疗可在一定程度上延缓肝脾病变的进展,定量影像学在戈谢病评估中具有一定的价值。Objective Quantitative imaging evaluation was performed on the liver and spleen system le⁃sions of patients with Gaucher disease after treatment.in order to deepen the understanding of Gaucher disease.Methods From August 1999 to August 2018,we registered,examined and treated children with Gaucher dis⁃ease,and conducted quantitative imaging research on 40 children with Gaucher disease who were intensively followed up in Beijing Children's hospital,Capital Medical University until August 2018.At the same time,34 normal volunteers were matched.All subjects were scanned with magnetic resonance imaging(MRI).The fat fraction(FF),iron content(R2^(∗)),standard apparent diffusion coefficient(sADC),slow apparent diffusion coefficient(D),fast apparent diffusion coefficient(D^(∗))and perfusion fraction(f)of the liver and spleen were measured.The quantitative parameter values measured by patients with Gaucher disease and normal subjects were statistically analyzed by independent sample t⁃tests.Results The results showed that there was no significant difference in FF,R2^(∗),sADC,D,D^(∗),f of the liver and spleen,and liver elasticity was also within the normal range.However,the volume of liver and spleen in patients was significantly different from that in normal subjects.Conclusions After treatment,the volume of the liver and spleen in patients with Gaucher disease is greater than that of normal people,but other quantitative parameters are within the normal range,indicating that long⁃term enzyme replacement therapy can delay the progress of liver and spleen diseases to a certain extent.Quantitative imaging has a certain value in the evaluation of Gaucher disease.

关 键 词：戈谢病 儿童 磁共振 

分 类 号：R445.2[医药卫生—影像医学与核医学] R589[医药卫生—诊断学]