Coagulation abnormalities in children with chronic liver disease and the association of these abnormalities with disease severity

Abstract

Background and aims

This study aimed to evaluate the coagulation profile in children with chronic liver disease (CLD) and to investigate the relationship between coagulation abnormalities and disease severity.

Methods

Fifty-one consecutive children (mean age 7.4 ± 4.9 years) diagnosed with CLD were prospectively included and stratified by the Child–Pugh class (A, B, C). Laboratory data, including the complete blood count, prothrombin time, activated partial thromboplastin time (APTT), international normalized ratio (INR), fibrinogen, D-dimer, and albumin-bilirubin (ALBI) score, were analyzed. Logistic regression identified predictors of the Child–Pugh class.

Results

Autoimmune hepatitis (31.3%) and Wilson's disease (27.4%) were the most common etiologies. Clinical features, such as gastrointestinal bleeding, ascites, and encephalopathy, were more common in Child–Pugh class C (p = 0.002). The INR (p = 0.017), the APTT (p = 0.018), and D-dimer concentrations (p = 0.001) increased with disease severity, while albumin concentrations decreased. The INR (OR: 86.8 for B vs A; OR: 82.4 for C vs A, p = 0.004) and hypoalbuminemia (OR: 0.11 for C vs A, p = 0.004) were the most robust independent predictors. D-dimer concentrations predicted advanced disease but lost significance after adjustment. The ALBI score was correlated with albumin, the INR, prothrombin time, the APTT, and D-dimer, while Child–Pugh score correlations were weaker. Fibrinogen concentrations were reduced in 64.7% of patients but not associated with severity.

Conclusion

The INR and albumin are reliable predictors of disease severity in pediatric CLD. The ALBI score provides more reproducible assessment of hepatic reserve than the Child–Pugh score and may be a useful tool for risk stratification in children.