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Role of plasma Von-Willebrand factor in children with chronic liver diseases
Ola Galal Ali Behairy1, Abdelhamid Mohamed El-Hamshary1, Ahmed Adel Torky2
1Pediatrics Department, Faculty of Medicine, Benha University, Benha, Egypt.
Insights
Von-Willebrand Factor Antigen (VWF Ag) plasma levels can predict liver fibrosis severity in children with chronic liver disease (CLD). Elevated VWF Ag levels accurately indicate mild and severe fibrosis, offering a non-invasive diagnostic approach.
Area of Science:
- Pediatric Gastroenterology and Hepatology
- Biomarker Discovery
- Non-invasive Diagnostics
Background:
- Liver biopsy (LB) is the gold standard for diagnosing and staging liver fibrosis in children with chronic liver diseases (CLD).
- Non-invasive methods for assessing liver fibrosis severity are crucial to reduce the need for invasive procedures.
- Von-Willebrand Factor Antigen (VWF Ag) has emerged as a potential non-invasive biomarker for liver fibrosis.
Purpose of the Study:
- To evaluate Von-Willebrand Factor Antigen (VWF Ag) plasma titer as a non-invasive predictor for grading liver fibrosis in pediatric CLD patients.
- To assess the correlation of VWF Ag levels with established fibrosis scores and clinical parameters.
- To determine the diagnostic accuracy of VWF Ag in identifying mild and severe liver fibrosis.
Main Methods:
- A case-control study involving 120 children (60 with CLD, 60 healthy controls).
- Diagnosis and staging of liver fibrosis were confirmed by liver biopsy (LB).
- VWF Ag plasma concentrations were measured using enzyme-linked immunosorbent assay (ELISA).
Main Results:
- Mean VWF Ag titers were significantly higher in children with CLD compared to healthy controls (581.4 ± 279 ng/ml vs. 166.9 ± 78 ng/ml; P < 0.001).
- VWF Ag showed a significant positive association with total bilirubin, direct serum bilirubin (DSB), AST, ALT, liver span, portal hypertension, MELD score, PELD score, and Child-Pugh score (P ≤ 0.05).
- VWF Ag demonstrated high accuracy in predicting mild (AUC 0.973) and severe fibrosis (AUC 0.988) with excellent sensitivity and specificity.
Conclusions:
- VWF Ag is a significant and reliable non-invasive indicator for assessing liver fibrosis severity in pediatric CLD.
- VWF Ag can be utilized as a valuable tool to complement or potentially reduce the reliance on liver biopsy for fibrosis staging in children.
- The findings support the use of VWF Ag as a promising biomarker in the management of pediatric chronic liver disease.
Abstract:
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.
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