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

慢性肝病患儿的凝血异常及其与疾病严重程度的关系

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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.

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