Ductular Reaction Underlies Development of Portal Hypertension in Biliary Atresia

ABSTRACT Background & Aims As better understanding of pathophysiology and reliable predictors for portal hypertension (PH) are needed, we investigated how histological ductular reaction (DR) and liver fibrosis predict PH in biliary atresia (BA). Methods 60 BA patients treated between 1987 and 2024 at Helsinki University Hospital were enrolled. Patients underwent liver biopsies at Kasai portoenterostomy and 1 year postoperatively. PH was defined as endoscopic detection of oesophageal varices or thrombocytopenia ( Results After portoenterostomy, PH was detected in 70% ( n  = 42) of patients at median age 1.4 years (IQR 0.7–2.6). At portoenterostomy, DR% ≥ 6.9% ( p  = 0.016) and PIH% ≥ 1.6% ( p  n  = 5) predicted PH ( p  = 0.032). At 1 year, DR% ≥ 3.5% ( p  p  p  = 0.006) as well as Metavir 3–4 vs. 1–2 ( p  = 0.037) and SRP% ≥ 28.4% ( p  = 0.006) predicted PH. Patients with PH showed progression of PIH% ( p   Conclusion DR predicted development of PH at and one year after portoenterostomy, associating with PH more consistently than fibrosis.