Biliary atresia in Turkish children

Area of Science:

• Pediatric Surgery
• Hepatology
• Neonatal Care

Background:

• Extrahepatic biliary atresia (EHBA) is a rare neonatal liver disease requiring prompt diagnosis and surgical intervention.
• Outcomes for EHBA patients in Turkey are influenced by diagnostic delays and limited organ availability.
• The Kasai procedure is a common surgical approach, but its success rates can vary.

Purpose of the Study:

• To assess the surgical success and survival rates of extrahepatic biliary atresia (EHBA) patients in Izmir, Turkey.
• To investigate the correlation between diagnosis/operation timing and patient outcomes.
• To identify factors influencing patient prognosis in EHBA.

Main Methods:

• Retrospective review of clinical and laboratory data for 27 EHBA patients.
• Analysis of 25 patients who underwent the Kasai procedure and 2 who received liver transplants.
• Definition of surgical success as jaundice clearance (bilirubin <2 mg/dL) and grouping based on disease progression and transplant timing.

Main Results:

• Median age at diagnosis was 63.5 days and at portoenterostomy was 67.5 days.
• The Kasai procedure was successful in only 8% of cases, with operations performed at a median of 35 and 42 days.
• Patients with progressive liver disease (Group II) had significantly higher rates of ascites, ALT, and globulin levels compared to Group I.

Conclusions:

• General practitioners must be vigilant in evaluating infants with prolonged jaundice in Turkey.
• Late diagnosis leading to cirrhosis at admission and low organ donation rates necessitate consideration of living-related liver transplantation.
• Timely intervention and evaluation for liver transplantation are critical for improving EHBA patient outcomes in the region.