Behairy Ola Galal Ali, El-Hamshary Abdelhamid Mohamed, Torky Ahmed Adel, Khashaba Rana Atef, Ali Basma Galal, Mohamed Nashwa Farouk
Pediatrics Department, Faculty of Medicine, Benha University, Benha, Egypt.
M.B.B.Ch, Faculty of Medicine, Cairo University, Menoufia, Cairo, 32861, Egypt.
Eur J Pediatr. 2025 Aug 5;184(8):532. doi: 10.1007/s00431-025-06355-7.
Children with chronic liver diseases (CLD) face more significant clinical problems. The most reliable technique for detecting liver fibrosis is still liver biopsy (LB). Our investigation assessed Von-Willebrand Factor Antigen (VWF Ag) plasma titer as a probable non-invasive predictor for grading liver fibrosis in children with CLD. 120 children participated in our case-control study, 60 of whom had CLD and the remaining 60 of whom were healthy. Underlying etiologies were diagnosed via clinical, biochemical, and histological criteria by LB. VWF Ag concentrations were determined in all participants using enzyme-linked immunosorbent assay (ELISA). VWF Ag mean titers were significantly elevated in CLD cases in contrast with controls (581.4 ± 279 vs. 166.9 ± 78 ng/ml; P < 0.001). There was a remarkable positive association amongst VWF Ag and total, direct serum bilirubin (TSB, DSB), aspartate aminotransferase (AST), alanine aminotransferase (ALT), liver span, portal hypertension, Model for End-Stage Liver Disease (MELD) score, Pediatric End-Stage Liver Disease (PELD) score, and Child-Pugh score (P ≤ 0.05). VWF Ag anticipated mild (AUC 0.973, cutoff > 266.5 ng/ml) and severe fibrosis (AUC 0.988, cutoff > 590.3 ng/ml) with sensitivity and specificity of 100% and 86.7% for mild fibrosis and 100% and 92.9% for severe fibrosis, respectively.
VWF Ag can serve as a significant indicator of liver fibrosis severity in pediatric CLD cases.
• Liver biopsy remains the cornerstone for diagnosing and staging liver fibrosis in children.
• VWF Ag is a promising non-invasive indicator for grading liver fibrosis severity in pediatric chronic liver disease. • VWF Ag correlates strongly with fibrosis scores as Child-Pugh, MELD, PELD, AST/PLT ratio index (APRI), and fibrosis-4 (FIB-4), and histological fibrosis stages (by ISHAK score) in children.
患有慢性肝病（CLD）的儿童面临更严重的临床问题。检测肝纤维化最可靠的技术仍然是肝活检（LB）。我们的研究评估了血管性血友病因子抗原（VWF Ag）血浆滴度作为CLD儿童肝纤维化分级的一种可能的非侵入性预测指标。120名儿童参与了我们的病例对照研究，其中60名患有CLD，其余60名健康。通过临床、生化和LB组织学标准诊断潜在病因。使用酶联免疫吸附测定（ELISA）测定所有参与者的VWF Ag浓度。与对照组相比，CLD病例的VWF Ag平均滴度显著升高（581.4±279对166.9±78 ng/ml；P<0.001）。VWF Ag与总胆红素、直接血清胆红素（TSB、DSB）、天冬氨酸转氨酶（AST）、丙氨酸转氨酶（ALT）、肝上下径、门静脉高压、终末期肝病模型（MELD）评分、儿童终末期肝病（PELD）评分和Child-Pugh评分之间存在显著正相关（P≤0.05）。VWF Ag预测轻度纤维化（AUC 0.973，临界值>266.5 ng/ml）和重度纤维化（AUC 0.988，临界值>590.3 ng/ml），轻度纤维化的敏感性和特异性分别为100%和86.7%，重度纤维化为100%和92.9%。
VWF Ag可作为小儿CLD病例肝纤维化严重程度的重要指标。
•肝活检仍然是儿童肝纤维化诊断和分期的基石。
•VWF Ag是小儿慢性肝病肝纤维化严重程度分级的一个有前景的非侵入性指标。•VWF Ag与儿童的Child-Pugh、MELD、PELD、AST/血小板比值指数（APRI）和纤维化-4（FIB-4）等纤维化评分以及组织学纤维化阶段（通过ISHAK评分）密切相关。
