Brain MRI imaging markers associated with death in children with central nervous system involvement of hemophagocytic lymphohistiocytosis

作者全名："Ma, Wei; Zhou, Liang; Li, Wei; Li, Xiujuan; Huang, Yan; Gao, Sijie; Yu, Jie; Fang, Yuan; Xu, Ye"

作者地址："[Ma, Wei; Li, Wei; Gao, Sijie; Xu, Ye] Chongqing Med Univ, Natl Clin Res Ctr Child Hlth & Disorders,Children, China Int Sci & Technol Cooperat Base Child Dev &, Dept Radiol,Minist Educ,Key Lab Child Dev & Disor, 136 Zhongshan Er Lu, Chongqing 400000, Peoples R China; [Ma, Wei; Huang, Yan; Fang, Yuan] Peoples Hosp Yubei Dist Chongqing City, Dept Radiol, Chongqing 400000, Peoples R China; [Zhou, Liang] Chongqing Med Univ, Dept Emergency, Childrens Hosp, Chongqing 400000, Peoples R China; [Li, Xiujuan] Chongqing Med Univ, Dept Neurol, Childrens Hosp, Chongqing 400000, Peoples R China; [Yu, Jie] Chongqing Med Univ, Dept Hematol, Childrens Hosp, Chongqing 400000, Peoples R China"

通信作者："Xu, Y (通讯作者)，Chongqing Med Univ, Natl Clin Res Ctr Child Hlth & Disorders,Children, China Int Sci & Technol Cooperat Base Child Dev &, Dept Radiol,Minist Educ,Key Lab Child Dev & Disor, 136 Zhongshan Er Lu, Chongqing 400000, Peoples R China."

来源：EUROPEAN RADIOLOGY

ESI学科分类：CLINICAL MEDICINE

WOS号：WOS:001108443200007

JCR分区：Q1

影响因子：4.7

年份：2023

卷号：　

期号：　

开始页：　

结束页：　

文献类型：Article; Early Access

关键词：Child; Hemophagocytic lymphohistiocytosis; Central nervous system; Magnetic resonance imaging; Risk factors

摘要："Objectives To investigate the association of brain MRI and clinical variables with death in children with central nervous system involvement of hemophagocytic lymphohistiocytosis (CNS-HLH). Methods Clinical and brain MRI data of children with CNS-HLH from January 2012 to March 2022 were reviewed retrospectively. Patients were divided into the deceased group and the surviving group. The intergroup differences of seven brain MRI variables, twelve clinical variables, and underlying diseases were studied. Results One hundred and fourteen patients were included in this study, consisting of 59 who died and 55 who survived. The included clinical variables did not show statistically independent correlation with patients' deaths. For MRI variables, a multivariate analysis demonstrated restricted diffusion of lesion (OR=9.64, 95% CI: 3.39-27.43, p<0.001) and count of affected brain regions (CABR) (OR=1.24, 95% CI: 1.03-1.49, p=0.02) were independent risk factors for death. ROC curve showed CABR (AUC=0.79, 95% CI: 0.70-0.87, p<0.001) is highly predictive for mortality with an optimal cutoff value of 4.5 (sensitivity 76%, specificity 73%). For HLH subtypes, familial HLH (F-HLH, OR=9.90, 95% CI: 2.01-48.87, p=0.005) and immune-compromise-related HLH (IC-HLH, OR=4.95, 95% CI: 1.40-17.46, p=0.01) presented statistically stronger association with death than infection-related HLH. F-HLH and IC-HLH preferred to have large lesions, restricted diffusion, and more brain regions involved than other subtypes. Conclusion Brain MRI features exhibit independent prediction for mortality in children with CNS-HLH, and HLH subtypes pose effects on patient outcomes and brain MRI findings."

基金机构："National Natural Science Foundation of China [81301300]; Chongqing Science and Technology Foundation; Chongqing Science and Technology Commission [cstc2018jscx-msybX0069, cstc2016 shmszx130009]"

基金资助正文："This study has received funding from the National Natural Science Foundation of China (81301300), Chongqing Science and Technology Foundation, and Chongqing Science and Technology Commission (cstc2018jscx-msybX0069 and cstc2016 shmszx130009)."