ĐẶC ĐIỂM XƠ GAN, BIẾN CHỨNG VÀ TỬ VONG CỦA BỆNH NHÂN TEO ĐƯỜNG MẬT CÓ CHỈ ĐỊNH GHÉP GAN TẠI BỆNH VIỆN NHI ĐỒNG 2

Abstract

Introduction: Biliary atresia (BA) is a most common cause of cholestatic jaundice in neonates and infants, accounting for 25–40% of cases. After Kasai surgery, one-third of children with BA require liver transplantation (LT) within 24 months, and 80% within 20 years. BA-related indications account for 50% of pediatric LT transplants. In Vietnam, the number of children currently indicated for LT remains high, with the majority of cases being BA. This study was conducted to analyze the group of BA patients awaiting LT. Patients and Methods:This is a prospective, descriptive-analytical study. All BA patients indicated for LT at Children’s Hospital 2 were enrolled and followed from May 2023 to August 2024. Important variables included epidemiological, clinical, and laboratory characteristics, severity and complications of chronic liver disease, and patient outcomes (either transplanted or mortality). Results: A total of 99 BA cases indicated for LT were included in the study. Most patients were classified as Child-Pugh B and C (94.9%). The median PELD score was 17 (range: 13–22). The proportion of patients with PELD ≥ 10 or MELD ≥ 15 was 88.9%. The most common complication was portal hypertension, developed in 83.8% of cases (83/99), with gastrointestinal bleeding occurring in 27.7% of those (23/83). Cholangitis occurred in 60.6% of cases (60/99). During the study, 16/97 patients (16.5%) died, including one who died post-transplant. Only 10 patients (10.1%) underwent a LT. Factors associated with mortality included: younger age, non-Kasai, ascites, failed bile drainage, low GGT, elevated bilirubin, low albumin, increased INR, high Child-Pugh scores, Child-Pugh class C, high PELD scores, weight-for-age <–2 SD, and severe acute malnutrition. Among these, the non- Kasai was the only independent prognostic factor for mortality. Conclusion: Children with BA indicated for LT was a critically ill population with multiple complications. Most had high PELD or MELD scores and were classified as Child-Pugh B or C. The mortality rate was 16.5%, while the transplant rate remained low at only 10.1%. Low GGT levels being associated with mortality was a new and noteworthy finding in BA patients in need of LT.