目的 基于临床和CT血管造影(CTA)特征分析儿童多发性大动脉炎(TA)并发心功能不全(CD)的危险因素.方法 回顾性分析39例T A患儿,男9例、女30例,年龄1个月～15岁、中位年龄8岁.39例中,7例伴CD(CD组),均为女性,年龄2～15岁、中位年龄13岁;32例无CD(无CD组),男9例、女23例,年龄1个月～14岁、中位年龄7岁;对比分析2组患儿性别、年龄及主动脉受累部位等.结果 2组患儿年龄分布范围差异有统计学意义(P<0.05).高血压、升主动脉受累是TA患儿伴发CD的独立危险因素(P均<0.05),高血压及升主动脉受累TA患儿发生CD的概率分别为非高血压及无升主动脉受累者的14.493和13.889倍.结论 高血压及升主动脉受累均为T A患儿伴发CD的独立危险因素,应积极予以干预.

Objective:To investigate imaging features and clinical diagnostic value of pyogenic sacroiliitis (PS) in children.Methods:A total of 7 children with PS admitted to Children′s Hospital, Captial Institute of Pediatrics from January 2016 to June 2021 were selected into this study. The clinical data, X-ray images result of sacroiliac joint, CT and MRI findings of these 7 PS children were analyzed retrospectively. The study was conducted in accordance with World Medical Association Declaration of Helsinki revised in 2013. Informed consent was obtained from each participate′ guardian.Results:① These 7 PS children aged from 6 to 14 years old, including 4 boys and 3 girls, were involved in unilateral PS. ②Only one case (children No.1) had an abnormality suggested by X-ray orthopantomogram of the coarse articular surface of iliac crest. ② CT images showed sacroiliac joint space narrowing in 2 cases (children No.1, 7) and articular surface destruction in 6 cases (children No.1-3, 5-7), 5 (children No. 1-3, 5, 6) of which were located in ilium surface. Euphotic zone under articular surface of iliac was seen in 3 cases (children No.1, 2, 5). In 3 cases (children No. 1, 2, 7), there was soft tissue swelling around sacroiliac joint. One (child No.2) of the 2 cases (children No.1, 2 ) performed enhanced CT showed abscess formation. ④The results of MRI showed that no definite sacroiliac joint stenosis was found in 7 children, and high signal shadow of fat-suppression protein density weighted imaging(FS PDWI) in sacroiliac joint space were seen in 6 children (children No.2-7), and 5 cases (children No.1, 2, 5-7) showed articular surface of sacroiliac joint destruction. Among 6 children (children No.1, 2, 4-7) with bone marrow involvement in bilateral articular surfaces of sacroiliac joint, only one child (child No.7) was located on sacrum side, and the rest were located on ilium side. Soft tissue of sacroiliac joint abscess occurred in 4 cases (children No.2, 5-7). FS PDWI of MRI showed that there was a round-like high signal focus in the diffuse high signal area, with clear edge and partial wall structure. After enhancement, annular enhancement of MRI could be seen in T1WI images, and 4 cases (children No.1, 2, 5, 6) showed signs of lymph node enlargement around sacroiliac joint.Conclusions:The clinical signs of PS in children lack of specificity and can be easily misdiagnosed. MRI has high sensitivity and specificity to auxiliary diagnosis of PS, it can fully evaluate sacroiliac joint inflammation, bone marrow edema under the sacroiliac joint and soft tissue swelling of sacroiliac. It has high detection rate of abscess and it′s the first choice for early diagnosis of this disease, which is better than X-ray and CT. CT can show the bone changes in progressive stage of lesions clearly (including articular surface bone destruction and joint space stenosis), which is helpful to judge prognosis of children, and is an important complementary imaging method for MRI examination.

目的 观察幼年特发性关节炎(JIA)累及踝部的MRI表现.方法 回顾性观察42例JIA患儿受累踝部的MRI资料,分析其特点.结果 JIA共累及46侧踝部,38例单侧、4例双侧踝部受累.①37侧(37/46,80.43％)踝见关节滑膜炎,累及28个(28/43,65.12％)胫距关节、10个(10/43,23.26％)距舟关节及5个(5/43,11.63％)跟距关节;②26侧(26/46,56.52％)踝见关节积液,多累及胫距关节(19/26,73.08％),且均合并同部位滑膜炎;③26侧踝可见腱鞘炎(26/46,56.52％),多累及内踝组(15/30,50.00％)或外踝组(11/30,36.67％),前踝组较少见(4/30,13.33％),其中22侧(22/26,84.62％)合并滑膜炎、4侧(4/26,15.38％)表现为单一腱鞘炎;④15侧(15/46,32.61％)踝见骨髓水肿样改变,均累及多骨骼,常见受累部位依次为距骨(13/31,41.94％)、跟骨(10/31,32.26％)、胫骨远端(8/31,25.81％);其中11侧(11/15,73.33％)病变位于关节面下,以宽基底与关节面相连,且均合并邻近关节滑膜炎,4侧(4/15,26.67％)未伴发滑膜炎或腱鞘炎病变均远离关节面;⑤13侧踝见软组织肿胀(13/46,28.26％),分别见于内踝(7/13,53.85％)、外踝(5/13,38.46％)或内外踝同时受累(3/13,23.08％).结论 JIA累及踝部的最主要MRI表现包括滑膜炎、关节积液和腱鞘炎,且腱鞘炎可作为唯一受累征象出现;骨髓水肿样改变常见,需与生理性表现相鉴别.

目的 采用高分辨率CT(HRCT)观察不同来源干细胞移植治疗B型尼曼-皮克病(NPD)患儿肺间质病变的效果.方法 回顾性分析6例B型NPD患儿接受造血干细胞移植前后肺部HRCT资料,其中3例接受非亲缘脐带血干细胞移植治疗,3例接受亲缘单倍体骨髓+外周血干细胞移植治疗;于3个层面(主动脉弓层面、气管隆嵴层面及右膈顶层面)观察移植前后肺间质病变(表现为小叶间隔增厚、小叶中心阴影和小叶内线3种CT征象)累及范围并进行评分,计算各征象评分变化速率及征象评分均值变化速率.结果 接受干细胞移植后,6例患儿肺间质病变评分均较移植前呈明显下降趋势;其中3例接受非亲缘脐带血干细胞移植者肺部小叶间隔增厚、小叶中心阴影和小叶内线的征象评分变化速率及征象评分均值变化速率平均值(1.16分/月、1.33分/月、1.11分/月、1.24分/月)均高于3例接受亲缘单倍体骨髓+外周血干细胞移植者(1.14分/月、0.86分/月、0.88分/月、1.03分/月).结论 干细胞移植可改善B型NPD患儿肺间质病变;非亲缘供体脐带血干细胞移植可能较亲缘单倍体移植治疗效果更佳.

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 (χ2=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. The linear regression equation between the score of airway, interstitial, alveolar lesions and the HSCT duration was: airway lesion, Y=1.94-0.15X; interstitial lesion, Y=12.73-0.78X; and alveolar lesion, Y=3.31-0.27X. The results of the generalized linear mixed model showed that the main effect of HSCT duration significantly affected on the three kinds of lung lesions, and the scores of interstitial lesions, airway lesions and alveolar lesions were decreased by 0.688, 0.245, and 0.338 points for each 1 month increase of HSCT duration (all P<0.05). The alveolar lesion score decreased by 1.135 points for each 1 year increase in age at presentation ( P=0.012). The main effects of gender and co-infection were not statistically significant in all of the pulmonary lesions (both P>0.05). Conclusion:HSCT alleviates pulmonary lesions of children with NPD type B significantly and consistently, with interstitial lesions were the most prominent.

目的 观察婴幼儿尼曼-皮克病肺部病变的高分辨率C T(H RC T)表现.方法 回顾性分析11例经基因检测/骨髓穿刺活检/酶学活性检测确诊尼曼-皮克病患儿的胸部H RC T表现,包括气道病变征象、间质病变征象、肺泡病变征象及其他肺或肺外改变.对双肺主动脉弓层面、气管隆嵴层面及膈面上层面的H RC T异常及累及范围分别进行评分.比较不同种类病变及累及范围的差异.结果 气道病变和间质病变发生率均为100％(11/11),肺泡受累为81.82％(9/11);上述3类病变累及范围差异有统计学意义(H=10.57,P<0.01),而左、右肺野病变累及范围相近(U=27.5,P=0.64).气道病变所致各种异常累及范围差异有统计学意义(H=8.81,P=0.03),以支气管壁增厚和树芽征范围最广.肺间质病变所致小叶间隔增厚、小叶内线及小叶中心阴影累及范围差异无统计学意义(H=5.67,P=0.06).9例患儿肺部见磨玻璃影,累及范围相近.结论 尼曼-皮克病肺部病变H RC T表现以肺间质和肺泡受累为主,间质病变多见于上肺野和下肺野,气道病变多见于上肺野,而肺泡病变分布较广泛、均匀.

目的:探讨CT能谱成像(GSI)技术在学龄儿童腹部血管成像中的最佳单能量图像.方法:选取我科腹部增强CT扫描的学龄期儿童30例,男19例,女11例,平均年龄(9.4±2.8)岁,以GSI模式进行增强CT扫描.重建获得40～80 KeV单能量图像41组及140 KvP混合能量图像.分别测量腹主动脉、门静脉、下腔静脉、竖脊肌CT值.分析图像CT值与能量变化关系,测量图像CNR及噪声,对最高CNR与最低噪声单能量图像以及混合能量图像进行主观评分.对数据进行统计学分析.结果:CNR变化曲线总体呈双峰下降趋势,峰值分别位于40 keV与60～65 keV.门静脉、腹主动脉、下腔静脉的最佳CNR分别位于(62.0±2.1),(61.4±1.9)和(60.7±2.0) keV.噪声最低的单能量图像为68 keV.60～62 keV单能量图像主观评分(10.2±0.6;10.2±0.8;10.2±0.8)高于混合能量图像(9.1±0.6),其差异具有统计学意义(P＜0.001).结论:学龄儿童腹部血管成像,观察腹主动脉、门静脉、下腔静脉的最佳单能量图像为60～62 KeY.

目的 探讨CT能谱成像(GSI)技术在学龄儿童腹部增强检查中的最佳单能量图像.方法 搜集2015年2月至7月于本院行GSI腹部增强CT扫描的学龄期儿童38例,男22例,女16例,其中最小年龄6.0岁,最大年龄14.0岁,平均(8.7±3.9)岁.以GSI模式进行增强CT扫描.注药60s后进行扫描.用标准重组模式GSI处理/重组平台重组图像,获得41组单能量图像(从40 keV至80 keV,间距1 keV)及140 kVp混合能量图像.对肝、脾与竖脊肌进行CT值测量,首先评价客观指标评价,包括图像信噪比(SNR)及噪声;然后在最高SNR单能量图像以及混合能量图像中进行主观指标评价,包括脏器显示、肌肉脂肪间隙显示及噪声,均分为4个等级,得出图像质量评分.对所有数据进行统计学分析.结果 肝、脾的SNR随能量变化的趋势一致,在40 ～ 55 keV时呈平直线,后逐渐增高,至65 keV时达到高峰,随后SNR逐渐下降,止于40 keV时水平.噪声在40 keV时出现最高值,后逐渐下降,在54 keV时下降幅度增大,在68 keV时达到最低值后缓步上升,其总体变化呈下降趋势.65 keV单能量图像主观评分优于混合能量图像,分值差异具有统计学意义(t=20.29,P＜0.001).结论 学龄儿童腹部GSI增强检查中,观察肝、脾的最佳单能量图像为65 keV,噪声最低的最佳单能量图像为68 keV.