造血干细胞移植对儿童尼曼-皮克病B型肺部病变影响的高分辨CT表现  

The influence of hematopoietic stem cell transplant on pulmonary involvement in pediatric type B Niemann-Pick disease:high resolution CT findings

作　　者：辛海燕[1] 陈瑞 孟玲慧[2] 闫淯淳 刘嵘[3] 袁新宇[1] Xin Haiyan;Chen Rui;Meng Linghui;Yan Yuchun;Liu Rong;Yuan Xinyu(Department of Radiology,Children′s Hospital Affiliated to Capital Institute of Pediatrics,Beijing 100020,China;Department of Medical Statistics,Capital Institute of Pediatrics,Beijing 100020,China;Department of Hematology,Children′s Hospital Affiliated to Capital Institute of Pediatrics,Beijing 100020,China)

机构地区：[1]首都儿科研究所附属儿童医院放射科,北京100020 [2]首都儿科研究所医学统计室,北京100020 [3]首都儿科研究所附属儿童医院血液科,北京100020

出　　处：《中华放射学杂志》2022年第4期431-436,共6页Chinese Journal of Radiology

摘　　要：目的分析6例尼曼-皮克病(NPD)B型患儿造血干细胞移植(HSCT)治疗前后的胸部高分辨CT(HRCT)图像,探讨HSCT对肺部病变的影响。方法回顾性收集2019年3月至2021年6月首都儿科研究所附属儿童医院临床确诊的6例NPD B型并进行HSCT治疗的患儿资料,男5例、女1例,就诊年龄为1岁2个月至5岁,中位年龄2岁1个月。HSCT后HRCT随访时间为7~20.5个月,中位时间5个月,随访次数2~7次/例,共27次。由2名医师在主动脉弓层面、气管隆突层面、右膈面上层面对肺部病变(肺间质病变、气道病变、肺泡病变)分别进行评估和计分,取两者均值为最终评分。采用Kruskal-WallisH检验对HSCT前3种肺部病变评分进行比较;采用线性回归的方法分析不同病变变化程度随HSCT时长的变化特点;采用广义线性混合模型探讨HSCT时长、就诊年龄、性别以及是否合并感染对HSCT后不同种类病变的影响。结果在HSCT治疗前,本组6例患者均均存在不同程度气道、间质和肺泡病变,中位评分分别为3.0、14.0、5.8分,差异有统计学意义(χ^(2)=11.95,P=0.003),其中以肺间质病变最广泛和最严重。经HSCT治疗后,6例患儿的肺间质和气道病变评分均随HSCT后时间延长呈不同程度下降,除病例4外,5例患儿肺泡病变评分亦随时间推移呈下降趋势。气道病变、肺间质病变及肺泡病变评分与HSCT时长的线性方程为:气道病变,Y=1.94-0.15X;间质病变,Y=12.73-0.78X;肺泡病变,Y=3.31-0.27X。广义线性混合模型结果显示,移植时长的主效应对于3种肺部病变均有显著影响,HSCT时长每增加1个月,间质病变、气道病变、肺泡病变评分分别平均下降0.688、0.245和0.338分(P均<0.05);就诊年龄每增加1岁,肺泡病变评分平均降低1.135分(P=0.012),而性别和是否合并感染的主效应对肺部病变均无显著影响(P均>0.05)。结论HSCT可明显而持续改善儿童NPD B型病例的肺部病变,且以肺间质病变的吸收最快、�Objective To analyze the pulmonary high resolution CT(HRCT)images before and after hematopoietic stem cell transplantation(HSCT)in 6 children with Niemann-Pick disease(NPD)type B,and to investigate the effect of HSCT on lung lesions.Methods Data of 6 children who were diagnosed with NPD type B and underwent HSCT treatment in Children′s Hospital Affiliated to Capital Institute of Pediatric from March 2019 to June 2021 were retrospectively enrolled,including 5 males and 1 female,with ages ranging from 1 year and 2 months to 5 years,and a median age of 2 years and 1month.The follow-up time of HRCT after HSCT was 7-20.5 months,the median time was 5 months,and the number of follow-up was 2-7 per patients,a total of 27 times.The pulmonary lesions(including interstitial lung disease,airway lesions and alveolar lesions)on aortic arch level,tracheal carina level and right diaphragmatic surface level were evaluated and scored respectively by two experienced pediatric radiologists,and the average score between them would be the final score.The Kruskal-Wallis H test was used to compare the scores of the three kinds of lung lesions before HSCT.The linear regression method was used to analyze the impact of HSCT duration on the degree of different lesions.To control possible confounding factors in the study,a generalized linear mixed model was used to evaluate the effects of HSCT duration,age,gender and whether co-infection on different types of lesions after HSCT.Results Before HSCT treatment,all of the 6 children had different degree of airway,interstitial and alveolar lesions,with a median score of 3.0,14.0 and 5.8 points,respectively(χ^(2)=11.95,P=0.003).Interstitial disease was the most extensive and serious lesion among those three pulmonary involvements in pediatric NPD type B.After HSCT treatment,the scores of interstitial and airway lesions in all of the 6 children reduced,in varying degree,with the increase of time after HSCT.Except case 4,the alveolar lesion in 5 patients also showed a decreasing trend over time.T

关 键 词：尼曼-皮克病 B型 造血干细胞移植 儿童 肺 体层摄影术 X线计算机 

分 类 号：R725.6[医药卫生—儿科] R457.7[医药卫生—临床医学]