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.Conclusion: VWF Ag can serve as a significant indicator of liver fibrosis severity in pediatric CLD cases.<Table Float="No" ID="Taba"> <tgroup cols="1"> <colspec align="left" colname="c1" colnum="1" /> <tbody> <row> <entry align="left" colname="c1"> What is Known: • Liver biopsy remains the cornerstone for diagnosing and staging liver fibrosis in children. </entry> </row> <row> <entry align="left" colname="c1"> What is New: • 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. </entry> </row> </tbody> </tgroup> </Table>

Role of plasma Von-Willebrand factor in children with chronic liver diseases

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2025年8月5日

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Ola Galal Ali Behairy,
Abdelhamid Mohamed El-Hamshary,
Ahmed Adel Torky,
Rana Atef Khashaba,
Basma Galal Ali,
Nashwa Farouk Mohamed

摘要

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.

Conclusion: VWF Ag can serve as a significant indicator of liver fibrosis severity in pediatric CLD cases.

What is Known:

• Liver biopsy remains the cornerstone for diagnosing and staging liver fibrosis in children.

What is New:

• 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.