Portal hypertension in biliary atresia: a Japanese biliary atresia registry study
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Purpose
This study aimed to clarify the incidence of portal hypertension (PH) in biliary atresia (BA), its impact on long-term native liver survival rates (NLSRs), and the role of PH treatment.
Methods
Data were analyzed from 3,777 patients in the Japanese Biliary Atresia Registry (1989–2021). Incidence and treatment of PH were examined in all patients and in 20-year native liver survivors. A subgroup of 596 jaundice-free native liver survivors was divided into three groups: without PH (A), untreated PH (B), and treated PH (C). Patient characteristics, clinical outcomes, and survival analyses were performed.
Results
PH occurred in 46.4% of all patients, with gastroesophageal varices (35.1%) and hypersplenism (36.8%) being most common. Among 759 20-year native liver survivors, 49.8% had PH, largely diagnosed between 2 and 15 years. Group C patients were older at Kasai portoenterostomy and had more cholangitis. Survival analysis showed best outcomes in group A and worst in group C. Conditional native liver survival analysis revealed group B initially had better NLSRs than group C, but differences diminished beyond 15 years.
Conclusion
In native liver survivors without jaundice following KP, PH influenced prognosis, but successful treatment enabled long-term survival comparable to patients with mild or no PH.
