Journal of Clinical Hepatology
This study retrospectively analyzed the clinical and pathological data of 26 patients with congenital liver fibrosis (CHF), systematically summarizing their clinical manifestations, laboratory indicators, and histological features across different age groups. The study is the first to compare liver function and blood routine changes by age stratification (children, adolescents, young adults, middle-aged and elderly), finding that enzyme indicators such as ALT, AST, and ALP show a decreasing trend with age, while globulin levels gradually increase, suggesting age-related progression of the disease. Combined with pathological images, the typical manifestations of CHF are identified as bile duct plate malformation and portal area fibrosis. Compared to previous studies that mainly focused on children or ARPKD-related cases, this study covers a wider age spectrum, filling the gap in clinical data for adult CHF and providing a basis for early diagnosis and individualized management.
This study enhances the understanding of the clinical heterogeneity of congenital liver fibrosis, particularly revealing the potential for covert progression of the disease in adults, which helps to avoid misdiagnosis. By refining biochemical characteristics across different age groups, it provides empirical support for clinicians to develop screening strategies and risk assessment models. In addition, the study emphasizes the critical role of pathological diagnosis, promoting collaboration between liver pathology and clinical medicine, and has guiding significance for improving the diagnosis and treatment pathway of CHF. As a large-scale single-center analysis in China, this achievement lays the foundation for subsequent multicenter studies and contributes data resources to the construction of the Chinese rare liver disease database.
Objectives: To summarize the clinical and pathological features of patients with congenital liver fibrosis (CHF), and to investigate…
