AIM:Biliary atresia (BA) is a neonatal liver disease defined as chronic progressive fibrotic obliteration of extrahepatic bile ducts. The objective of this study was to determine the association of serum connective tissue growth factor (CTGF) with clinical outcome and liver stiffness measurement. METHODS:Eighty-two BA patients post-Kasai operation and 28 healthy controls were recruited. BA patients were categorized into two groups based on their portal hypertension (PH) status. Serum CTGF levels were determined by enzyme-linked immunosorbent assay. Liver stiffness scores were measured by transient elastography. RESULTS:BA patients had greater CTGF levels (905.9 ± 57.7 vs 238.3 ± 23.5 pg/mL, P < 0.001) and higher liver stiffness values than controls (28.2 ± 2.6 vs 5.0 ± 0.5 kPa, P < 0.001). Serum CTGF levels were remarkably elevated in BA patients with PH compared to those without PH (1092.4 ± 73.9 vs 582.6 ± 45.7 pg/mL, P < 0.001). Furthermore, BA patients with PH had significantly higher liver stiffness values compared to those without PH (37.3 ± 3.0 vs 10.6 ± 1.1 kPa, P < 0.001). Additionally, serum CTGF was positively correlated with liver stiffness (r = 0.875, P < 0.001) and total bilirubin (r = 0.462, P < 0.001). There was an inverse correlation between serum CTGF and serum albumin (r = -0.579, P < 0.001). CONCLUSION:High serum CTGF was associated with a poor outcome in BA patients. Accordingly, serum CTGF and transient elastography may serve as non-invasive biomarkers reflecting the disease severity in postoperative BA patients.

译文

目的:胆道闭锁(BA)是一种新生儿肝病,定义为肝外胆管的慢性进行性纤维化闭塞症。这项研究的目的是确定血清结缔组织生长因子(CTGF)与临床结局和肝硬度测量之间的关系。
方法:招募Kasai手术后的82例BA患者和28例健康对照者。根据门脉高压(PH)的状态,将BA患者分为两类。通过酶联免疫吸附测定法测定血清CTGF水平。肝硬度评分通过瞬时弹性成像法测量。
结果:BA患者的CTGF水平较高(905.9±57.7 vs 238.3±23.5μpg/ mL,P <0.001),肝硬度值高于对照组(28.2±2.6和5.0±0.5 kPa,P 0.001)。与没有PH的BA患者相比,患有PH的BA患者的血清CTGF水平显着升高(1092.4±±73.9 vs 582.6±±45.7μpg/ mL,P <0.001)。此外,患有PH的BA患者的肝脏僵硬度值明显高于没有PH的患者(37.3±±3.0 kPa与10.6±±1.1±kPa,P <0.001)。另外,血清CTGF与肝硬度(r = 0.875,P <0.001)和总胆红素(r = 0.462,P <0.001)呈正相关。血清CTGF与血清白蛋白呈负相关(r = -0.579,P <0.001)。
结论:BA患者血清CTGF高与预后不良有关。因此,血清CTGF和瞬时弹性成像可以作为非侵入性生物标志物,反映术后BA患者的疾病严重程度。

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