Abdel Razek Ahmed Abdel Khalek, Barakat Tarik, Ali Khadiga
From the Department of Diagnostic Radiology.
Gastroenterology and Hepatology Unit of Mansoura Children Hospital, and.
J Comput Assist Tomogr. 2019 Mar/Apr;43(2):183-186. doi: 10.1097/RCT.0000000000000817.
The aim of this study was to assess of liver and spleen in children with Gaucher disease type 1 with chemical shift imaging.
Retrospective analysis was conducted on 36 untreated children with Gaucher disease type 1 and 15 age- and sex-matched control children who underwent chemical shift imaging of the abdomen. The hepatic and splenic fat fraction was calculated. The Pediatric Gaucher Severity Scoring System, hematologic parameters, and enzyme assay of the disease were calculated and correlated with hepatic and splenic fat fraction.
The mean hepatic fat fraction in Gaucher disease was 14.1 ± 3.1 (95% confidence interval [CI], 12.9-15.2) was significantly higher (P = 0.001) than that of control subjects 5.2 ± 0.9 (95% CI, 4.7-5.7). The mean splenic fat fraction in Gaucher disease was 11.2 ± 2.9 (95% CI, 11.2 ± 2.9), and that of control was 4.5 ± 0.9 (95% CI, 4.02-5.2). The thresholds of hepatic and splenic fat fraction used to differentiate patients from control subjects were 0.47 and 0.39 with areas under the curve of 0.947 and 0.999, respectively. The hepatic and splenic fat fraction is correlated with the Pediatric Gaucher Severity Scoring System (r = -0.523, -0.602), hemoglobin concentration (r = -0.491, -0.505), platelet count (r = -0.593, -0.24), β-glucosidase (r = 0.519, 0.549), and chitotriosidase (r = -0.03, 0.267), respectively.
Hepatic and splenic fat fraction is a promising quantitative imaging parameter for detection and quantification of hepatic and splenic infiltration in children with Gaucher disease and correlated with clinical scoring, hematologic parameters, and enzyme assay of the disease.
本研究旨在通过化学位移成像评估1型戈谢病患儿的肝脏和脾脏情况。
对36例未经治疗的1型戈谢病患儿及15例年龄和性别匹配的对照儿童进行回顾性分析，这些儿童均接受了腹部化学位移成像检查。计算肝脏和脾脏的脂肪分数。计算小儿戈谢病严重程度评分系统、血液学参数及该疾病的酶测定值，并将其与肝脏和脾脏脂肪分数进行相关性分析。
戈谢病患儿的平均肝脏脂肪分数为14.1±3.1（95%置信区间[CI]，12.9 - 15.2），显著高于对照组的5.2±0.9（95%CI，4.7 - 5.7）（P = 0.001）。戈谢病患儿的平均脾脏脂肪分数为11.2±2.9（95%CI，11.2±2.9），对照组为4.5±0.9（95%CI，4.02 - 5.2）。用于区分患者与对照的肝脏和脾脏脂肪分数阈值分别为0.47和0.39，曲线下面积分别为0.947和0.999。肝脏和脾脏脂肪分数分别与小儿戈谢病严重程度评分系统（r = -0.523，-0.602）、血红蛋白浓度（r = -0.491，-0.505）、血小板计数（r = -0.593，-0.24）、β-葡萄糖苷酶（r = 0.519，0.549）和壳三糖苷酶（r = -0.03，0.267）相关。
肝脏和脾脏脂肪分数是检测和量化戈谢病患儿肝脏和脾脏浸润的一个有前景的定量成像参数，且与临床评分、血液学参数及该疾病的酶测定值相关。
