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

Abstract

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

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.

Figures/Tables 3

References 19

[1]	中华医学会神经病学分会神经遗传病学组. 中国肝豆状核变性诊治指南[J]. 中华神经科杂志, 2021, 54(4): 310-319.
[2]	方峰. 儿童肝豆状核变性的长期治疗与随访管理[J]. 临床肝胆病杂志, 2017, 33(10): 1936-1938.
[3]	姜涛, 李双杰, 欧阳文献, 等. 330例遗传代谢性肝病患儿病因学分析[J]. 临床儿科杂志, 2021, 39(1): 6-8.
[4]	中华医学会肝病学分会, 中华医学会消化病学分会, 中华医学会感染病学分会. 肝纤维化诊断及治疗共识(2019)[J]. 中华肝脏病杂志, 2019, 27(9): 657-667.
[5]	张启迪, 陆伦根. 无创肝纤维化/肝硬化评估技术的现状[J]. 中华肝脏病杂志, 2018, 26(5): 325-327.
[6]	Brunt EM. Grading and staging the histopathological lesions of chronic hepatitis: The Knodell histology activity index and beyond[J]. Hepatology, 2000, 31 (1): 241-246. pmid: 10613753
[7]	Kim WR, Berg T, Asselah T, et al. Evaluation of APRI and FIB-4 scoring systems for non-invasive assessment of hepatic fibrosis in chronic hepatitis B patients[J]. J Hepatol, 2016, 64(4): 773-780. doi: 10.1016/j.jhep.2015.11.012
[8]	朱世殊, 董漪, 徐志强, 等. 以肝病为首发表现的儿童肝豆状核变性临床、病理及基因突变特征研究[J]. 传染病信息, 2013, 26(5): 272-275.
[9]	Roberts E A, Socha P. Wilson disease in children[J]. Handb Clin Neurol, 2017, 142: 141-156. doi: B978-0-444-63625-6.00012-4 pmid: 28433098
[10]	Schilsky ML. Liver transplantation for Wilson’s disease[J]. Ann N Y Acad Sci, 2014, 1315: 45-49. doi: 10.1111/nyas.12454
[11]	周丽娜, 林春, 潘晨, 等. 三种无创性肝纤维化评价方法在肝功能轻微异常的慢性HBV感染者中的诊断价值[J]. 中华临床感染病杂志, 2019, 12(4): 268-274.
[12]	仇丽霞, 梁珊, 范作鹏, 等. FibroScan对慢性丙型肝炎进展期肝纤维化的诊断价值及其影响因素[J]. 临床肝胆病杂志, 2019, 35(9): 1950-1953.
[13]	中国肝炎防治基金会, 中华医学会感染病学分会, 中华医学会肝病学分会和中国研究型医院学会肝病专业委员会. 瞬时弹性成像技术诊断肝纤维化专家共识(2018年更新版)[J]. 中华肝脏病杂志, 2019, 27(3): 182-191.
[14]	Marella HK, Reddy YK, Jiang Y, et al. Accuracy of non-invasive fibrosis scoring systems in African American and white patients with nonalcoholic fatty liver disease[J]. Clin Transl Gastroenterol, 2020, 11(4): e00165. doi: 10.14309/ctg.0000000000000165
[15]	Amernia B, Moosavy SH, Banookh F, et al. FIB-4, APRI, and AST/ALT ratio compared to FibroScan for the assessment of hepatic fibrosis in patients with non-alcoholic fatty liver disease in Bandar Abbas, Iran[J]. BMC gastroenterol, 2021, 21(1): 453. doi: 10.1186/s12876-021-02038-3 pmid: 34861841
[16]	Mesropyan N, Kupczyk P, Dold L, et al. Non-invasive assessment of liver fibrosis in autoimmune hepatitis: Diagnostic value of liver magnetic resonance parametric mapping including extracellular volume fraction[J]. Abdom Radiol (NY), 2021, 46(6): 2458-2466. doi: 10.1007/s00261-020-02822-x pmid: 33078245
[17]	梁晨, 刘晖, 白洁, 等. 常见临床指标及无创肝纤维化评分对中国肝病型Wilson 病进展期肝纤维化的诊断价值[J]. 临床肝胆病杂志, 2021, 37(3): 570-574.
[18]	Paternostro R, Pfeiffenberger J, Ferenci P, et al. Non- invasive diag nosis of cirrhosis and long-term disease monitoring by transient elastography in patients with Wilson disease[J]. Liver Int, 2020, 40(4): 894-904. doi: 10.1111/liv.14368 pmid: 31898387
[19]	Sini M, Sorbello O, Civolani A, et al. Non-invasive assessment of hepatic fibrosis in a series of patients with Wilson’s Disease[J]. Dig Liver Dis, 2012, 44(6): 487-491. doi: 10.1016/j.dld.2011.12.010

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

Value of noninvasive liver fibrosis evaluation in the diagnosis of advanced liver fibrosis in children with Wilson’s disease

RichHTML

PDF (PC)

Like

Knowledge

Abstract

Cite this article

share this article

Figures/Tables 3

References 19

Related Articles 15

Metrics

Comments

Recommended 0

[1]	ZOU Liping. Childhood encephalopathy: a group of diseases associated with various diseases [J]. Journal of Clinical Pediatrics, 2023, 41(9): 641-643.
[2]	ZHANG Weihua, ZOU Liping, REN Haitao, GUAN Hongzhi. Beware of the pitfalls in diagnosis and treatment of autoimmune encephalitis in children [J]. Journal of Clinical Pediatrics, 2023, 41(9): 644-649.
[3]	HOU Chi, CHEN Wenxiong, LIAO Yinting, WU Wenxiao, TIAN Yang, ZHU Haixia, PENG Bingwei, ZENG Yiru, WU Wenlin, CHEN Zongzong, LI Xiaojing. Clinical analysis of autoimmune glial fibrillary acidic protein astrocytopathy in children [J]. Journal of Clinical Pediatrics, 2023, 41(9): 656-660.
[4]	YANG Yating, CAI Yuehao, FANG Qiong, CHEN Lang, CHEN Qiaobin, LIN Zhi, WU Feifei, LIN Meng. Clinical analysis of idiopathic and symptomatic occipital lobe epilepsy in children [J]. Journal of Clinical Pediatrics, 2023, 41(9): 668-673.
[5]	HOU Ruolin, WU Jing, LI Ling. Pediatric autoimmune encephalitis with brain MRI showing meningeal thickening and enhancement [J]. Journal of Clinical Pediatrics, 2023, 41(9): 674-679.
[6]	WU Yuefang, SUN Yanling, WU Wanshui, DU Shuxu, LI Miao, SUN Liming. Analysis of prognostic factors and survival status of group 4 medulloblastoma in children [J]. Journal of Clinical Pediatrics, 2023, 41(9): 686-691.
[7]	SUN Juan, LI Haiying, JIA Peisheng, WANG Huaili. Clinical analysis of fulminant myocarditis in 12 children [J]. Journal of Clinical Pediatrics, 2023, 41(9): 692-696.
[8]	Reviewer: WANG Chenhui, Reviser: YANG Hui. Research progress on early screening and diagnosis of Crohn's disease in children [J]. Journal of Clinical Pediatrics, 2023, 41(9): 708-714.
[9]	SHEN Nan, DU Bailu. Strategies for the diagnosis, treatment, and management of invasive fungal infections in children with hematologic neoplasms [J]. Journal of Clinical Pediatrics, 2023, 41(8): 571-577.
[10]	XU Beixue, LIU Quanbo. Clinical analysis of 195 children with invasive pulmonary fungal infection [J]. Journal of Clinical Pediatrics, 2023, 41(8): 584-588.
[11]	CHEN Hongyu, LIU Zihao, WANG Heping, LIAO Cuijuan, LI Li, WANG Wenjian, LAI Jianwei. Role of nontypeable Haemophilus influenzae biofilms in chronic pulmonary infection in children [J]. Journal of Clinical Pediatrics, 2023, 41(8): 589-593.
[12]	KANG Lei, GUO Fang, LI Lifang, BAI Xinfeng, CHENG Caiyun, XU Meixian. Value of metagenomic next-generation sequencing in children with visceral leishmaniasis associated with hemolytic histiocytosis [J]. Journal of Clinical Pediatrics, 2023, 41(8): 594-598.
[13]	SUN Zhicai, LIU Yuling, LI Xiaolin, PAN Xiaofen. Clinical analysis of 15 children with primary nephrotic syndrome complicated with adrenal crisis [J]. Journal of Clinical Pediatrics, 2023, 41(8): 610-612.
[14]	WANG Hongxia, PAN Xiang, LU Jun. Report a case of α-ketoadipic aciduria caused by compound heterozygous variant of DHTKD1 gene [J]. Journal of Clinical Pediatrics, 2023, 41(8): 624-628.
[15]	XI Bixin, HU Qun, LIU Aiguo. Research advances of the bronchiolitis obliterans syndrome following allogeneic hematopoietic stem cell transplant in children [J]. Journal of Clinical Pediatrics, 2023, 41(8): 629-633.