9. Optimization of the management of patients with biliary atresia in the Republic of Kazakhstan: a clinical, epidemiological and prognostic study

Authors

  • N. Yerimova Syzganov National Scientific center of Surgery, Almaty, Kazakhstan
  • B. Baimakhanov Syzganov National Scientific center of Surgery, Almaty, Kazakhstan
  • B. Shirtayev Syzganov National Scientific center of Surgery, Almaty, Kazakhstan
  • B. Azadbekova Syzganov National Scientific center of Surgery, Almaty, Kazakhstan
  • D. Kurbanov Syzganov National Scientific center of Surgery, Almaty, Kazakhstan
  • M. Aitzhanov Syzganov National Scientific center of Surgery, Almaty, Kazakhstan
  • N. Rakhman Syzganov National Scientific center of Surgery, Almaty, Kazakhstan

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Abstract

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 RI Kazakhstan diagnostic delays remain D signiʖcant challenge QHFHVVLtating 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 ʖndings 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 days RI life signiʖcantly improved outcomes )actors associated with poor prognosis included elevated gamma-glutamyl transferase levels (above 600 international units per liter liver cirrhosis conʖrmed E\ biopsy mutations LQ the -$**(' gene, and altered expression of nuclear receptors-pregnane X receptor, constitutive androstane receptor. The predictive model incorporating these factors demonstrated 81 percent sensitivity and percent speciʖcity LQ 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. '2, %6. ,, Yerimova N.

Keywords

biliary, atresia, Kasai, portoenterostomy, liver, transplantation, Kazakhstan

References

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Published

2025-06-30

Issue

No. 2 (2025)

Section

Статьи