OPTIMIZATION OF THE MANAGEMENT OF PATIENTS WITH BILIARY ATRESIA IN THE REPUBLIC OF KAZAKHSTAN: A CLINICAL, EPIDEMIOLOGICAL AND PROGNOSTIC STUDY

Background. Biliary atresia is a rare, progressive disease of the bile ducts in infants (progressive cholangiopathy) that leads to cirrhosis and liver failure if untreated. Early diagnosis and timely surgical intervention are crucial for preserving the native liver. In the Republic of Kazakhstan, diagnostic delays remain a significant challenge, necessitating improved strategies for early detection and prognosis. Methods. A retrospective and prospective study was conducted involving 100 children diagnosed with biliary atresia. Patients were divided into two groups: those who underwent Kasaiportoenterostomy and those who required primary liver transplantation. Clinical characteristics, biochemical markers, ultrasound findings, and genetic data were collected. Statistical analysis included correlation studies, logistic regression, and receiver operating characteristic curve analysis to assess a predictive model for Kasai outcomes. Results. The Kasai procedure was successful in 34 percent of cases. Surgery performed before 60 days of life significantly improved outcomes. Factors associated with poor prognosis included elevated gamma-glutamyl transferase levels (above 600 international units per liter), liver cirrhosis confirmed by biopsy, mutations in the JAGGED1 gene, and altered expression of nuclear receptors-pregnane X receptor, constitutive androstane receptor. The predictive model incorporating these factors demonstrated 81 percent sensitivity and 76 percent specificity in identifying Kasai failure. Conclusion. Early intervention is critical to improve survival with the native liver in children with biliary atresia. The proposed prognostic model can support individualized treatment decisions. Implementation of a national screening and diagnostic algorithm in Kazakhstan could reduce delays and improve long-term outcomes.