无创肝纤维化评估在儿童肝豆状核变性进展期肝纤维化诊断价值

doi:10.12372/jcp.2022.21e1399

摘要/Abstract

摘要：

目的探讨临床生化指标、瞬时弹性成像技术及无创肝纤维化评分在肝豆状核变性（WD）儿童进展期肝纤维化诊断中的价值。方法收集2018年10月至2021年7月22例WD患儿的临床资料。患儿均行肝脏穿刺,采用瞬时弹性成像技术检测肝脏硬度值（LSM）。根据病理检查结果将患儿分为进展期肝纤维化（S3和S4期）和非进展期肝纤维化（S0、S1和S2期）两组,计算3种无创肝纤维化评分（Sheth、APRI和FIB-4指数）,比较两组间临床生化指标、无创肝纤维化评分和LSM的差异。采用受试者工作特征（ROC）曲线分析各指标对进展期肝纤维化的诊断价值。结果 22例WD患儿中,男12例、女10例,平均年龄（8.6±2.7）岁;非进展期肝纤维化为13例（59.1%）,进展期肝纤维化9例（40.9%）。与非进展期肝纤维化组相比,进展期肝纤维化组中白蛋白、天冬氨酸氨基转移酶（AST）和血小板（PLT）水平较低,凝血酶原时间（PT）和国际标准化比值（INR）水平较高,LSM、Sheth和FIB-4指数较高,差异均有统计学意义（P均<0.05）。ROC曲线分析提示,LSM（AUC=1.0）对进展期肝纤维化的诊断效能最大,其次为AST、PLT、PT、INR、FIB-4和Sheth指数（AUC均>0.7）。结论 AST、PLT、PT、INR、LSM、Sheth和FIB-4指数在儿童WD进展期肝纤维化中有一定的诊断价值,可以作为进展期肝纤维化的无创诊断参考指标。

关键词: 临床生化指标, 瞬时弹性成像, 无创肝纤维化评分, 肝豆状核变性, 儿童

Abstract:

Objective To investigate the value of clinical biochemical parameters, transient elastography and noninvasive liver fibrosis score in the diagnosis of advanced liver fibrosis in children with Wilson's disease (WD). Methods The clinical data of 22 WD patients from October 2018 to July 2021 were collected. All the children underwent liver biopsy, and the liver stiffness measurement (LSM) was measured by transient elastography. According to the pathological examination results, the children were divided into advanced liver fibrosis (S3 and S4) group and non-advanced liver fibrosis (S0, S1 and S2) group. Three kinds of non-invasive liver fibrosis scores (Sheth, APRI and FIB-4 index) were calculated, and the differences of clinical biochemical indexes, non-invasive liver fibrosis score and LSM between the two groups were compared. Receiver operating characteristic (ROC) curve was used to analyze the diagnostic value of each index for advanced liver fibrosis. Results Twenty-two WD children (12 boys and 10 girls) were included, with an average age of (8.6±2.7) years. There were 13 cases (59.1%) with non-advanced liver fibrosis and 9 cases (40.9%) with advanced liver fibrosis. Compared with non-advanced liver fibrosis group, the levels of albumin, aspartate aminotransferase (AST) and platelet (PLT) in advanced liver fibrosis group were lower, the prothrombin time (PT) and international standardized ratio (INR) were higher, and the indexes of LSM, Sheth and FIB-4 were higher, and the differences were significant (P<0.05). ROC curve analysis indicated that LSM (AUC=1.0) had the highest diagnostic efficacy for advanced liver fibrosis, followed by AST, PLT, PT, INR, FIB-4 and Sheth index (AUC>0.7). Conclusions AST, PLT, PT, INR, LSM, Sheth and FIB-4 index have certain diagnostic values in children with WD advanced liver fibrosis, and they can be used as a noninvasive diagnostic reference index in advanced liver fibrosis.

Key words: clinical biochemical parameter, transient elastography, noninvasive liver fibrosis score, Wilson’s disease, child

姜涛, 欧阳文献, 谭艳芳, 唐莲, 张慧, 李双杰. 无创肝纤维化评估在儿童肝豆状核变性进展期肝纤维化诊断价值[J]. 临床儿科杂志, 2022, 40(7): 534-538.

JIANG Tao, OUYANG Wenxian, TAN Yanfang, TANG Lian, ZHANG Hui, LI Shuangjie. Value of noninvasive liver fibrosis evaluation in the diagnosis of advanced liver fibrosis in children with Wilson’s disease[J]. Journal of Clinical Pediatrics, 2022, 40(7): 534-538.

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参考文献 19

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项目	非进展期肝纤维化组(n=13)	进展期肝纤维化组(n=9)	统计量	P
年龄(x±s)/岁	8.3±2.2	10.1±3.2	t=0.83	0.416
总蛋白(x±s)/g·L^-1	68.5±3.9	65.6±7.7	t=1.11	0.281
白蛋白(x±s)/g·L^-1	40.6±2.9	37.1±4.0	t=2.37	0.028
ALT[M(P₂₅~P₇₅)]/IU·L^-1	121.4(85.3~123.6)	102.1(51.9~182.5)	Z=0.23	0.815
AST[M(P₂₅~P₇₅)]/IU·L^-1	311.0(241.0~347.0)	197.0(158.0~220.0)	Z=3.11	0.002
WBC[M(P₂₅~P₇₅)]/×10⁹·L^-1	5.4(4.4~6.1)	5.5(4.7~7.4)	Z=0.30	0.764
中性粒细胞(x±s)/×10⁹·L^-1	2.1±0.6	2.6±0.9	t=1.37	0.187
淋巴细胞[M(P₂₅~P₇₅)]/×10⁹·L^-1	2.7(2.1~3.3)	2.7(2.1~3.0)	Z=0.30	0.764
Hb(x±s)/g·L^-1	126.0±11.4	122.0±10.1	t=0.83	0.417
PLT[M(P₂₅~P₇₅)]/×10⁹·L^-1	311.0(241.0~347.0)	197.0(158.0~220.0)	Z=3.11	0.002
PT[M(P₂₅~P₇₅)]/s	12.8±0.7	14.8±2.3	t=2.52	0.033
INR[M(P₂₅~P₇₅)]	1.0±0.1	1.2±0.2	t=2.50	0.034
APTT(x±s)/s	40.3±5.0	42.9±7.7	t=0.19	0.849
FIB(x±s)/mg·dL^-1	244.0±22.4	242.0±45.2	t=0.60	0.558
TT(x±s)/s	18.6±0.6	19.6±1.4	t=1.60	0.142

组别	例数	LSM（x±s）/kPa	Sheth指数[M(P₂₅~P₇₅)]	APRI指数（x±s）	FIB-4指数[M(P₂₅~P₇₅)]
非进展期肝纤维化组	13	6.3±1.0	0.5(0.4~1.3)	1.0±0.4	0.3(0.2~0.3)
进展期肝纤维化组	9	16.5±2.6	1.6(0.9~3.0)	1.6±0.7	0.7(0.3~1.2)
统计量		t=9.59	Z=2.17	t=1.98	Z=2.51
P		<0.001	0.030	0.074	0.012

变量	AUC (95%CI)	P	最佳临界值	灵敏度/%	特异度%
PT/s	0.86(0.67~1.00)	0.005	13.55	88.9	92.3
INR	0.85(0.67~1.00)	0.006	1.03	88.9	84.6
AST/IU·L^-1	0.90(0.75~1.00)	0.002	227.00	92.3	88.9
PLT/×10⁹·L^-1	0.90(0.75~1.00)	0.002	227.00	92.3	88.9
LSM/kPa	1.00(1.00~1.00)	<0.001	9.95	100.0	100.0
Sheth指数	0.78(0.57~0.98)	0.030	1.16	77.8	76.9
FIB-4指数	0.82(0.61~1.00)	0.012	0.51	66.7	100.0