Modern Pediatrics. Ukraine. (2025).7(151): 62-69. doi: 10.15574/SP.2025.7(151).6269
Kurylo H. V., Dubey L. Ya., Dubey N. V., Kotsai B. R.
Danylo Halytsky Lviv National Medical University, Lviv, Ukraine

For citation: Kurylo HV, Dubey LYa, Dubey NV, Kotsai BR. (2025). Cholestatic liver diseases in children: clinico-morphological features and strategies for optimizing treatment and transplantation. Modern Pediatrics. Ukraine. 7(151): 62-69. doi: 10.15574/SP.2025.7(151).6269.
Article received: Jul 27, 2025. Accepted for publication: Nov 09, 2025.

Cholestatic liver diseases in early childhood are characterized by rapid fibrosis progression, high risk of liver failure, and the need for timely surgical or transplant intervention. Early risk stratification requires integration of clinical, laboratory, morphological, and molecular markers.
Aim – to perform a comprehensive assessment of clinical, laboratory, morphological, and immunohistochemical features of cholestatic liver diseases in young children and to develop a pathogenetically justified algorithm for optimizing surgical and transplant management.
Materials and methods. Eighty-two children aged 1 month to 3 years with various forms of cholestatic liver disease were studied. Clinical evaluation, biochemical and coagulation tests, ultrasonography, elastography, and MRI were performed. Liver biopsy specimens were assessed using METAVIR and Ishak scoring systems; immunohistochemistry evaluated α-SMA, CK19, and Caspase-3 expression. Statistical analysis included parametric and non-parametric tests with significance set at p<0.05.
Results. Biliary atresia was the most common form of cholestasis, associated with earlier disease onset, higher direct bilirubin and γ-glutamyl transferase levels, and more frequent severe fibrosis (F3-F4) compared to other forms (p<0,05). Intrahepatic and genetic-metabolic forms showed slower morphological progression with predominantly mild fibrosis (F1-F2), although some patients exhibited high fibrogenic activity. Significant correlations were found between bile acids, direct bilirubin, alanine aminotransferase activity, and α-SMA expression (r=0.72-0,82; p<0.001). The integrated clinicopathological approach allowed optimization of surgical timing and transplant indications, accompanied by improvement in liver function tests (p<0,01).
Conclusions. Comprehensive evaluation of clinical, laboratory, morphological, and immunohistochemical parameters enables early identification of children at high risk of fibrosis progression, personalization of treatment, and reduction of early liver transplantation.
The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of the institution mentioned in the paper. Informed consent was obtained from the children's guardians for the study.
The authors declare no conflict of interest.
Keywords: cholestatic liver disease, early childhood, biliary atresia, Alagille syndrome, intrahepatic cholestasis, liver fibrosis, immunohistochemistry, liver transplantation.

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