Biliary atresia: early determination of prognosis

Analyses of bile bilirubin during the first month after Kasai operations, and of the liver biopsies obtained at the time of initial surgery, were done in 67 patients with biliary atresia. Bilirubin excretion (milligrams per day) was determined as the product of the bile volume and its concentration. Operative liver biopsies were evaluated for fibrosis, bile duct proliferation, bile stasis, giant cell transformation, and parenchymal degeneration; the severity of each abnormality was graded on a scale of 0 to 4. A forward stepwise regression procedure using the Cox proportional hazards model identified the relationship between survival and covariants. Thirty-nine of 67 patients died. Of these, 38 excreted less than 6 mg of bilirubin per day during the first postoperative month. The other patient died of a coexisting anomaly. Nine other patients who excreted less than 6 mg of bilirubin per day are alive but are either jaundiced or awaiting transplantation. Nineteen patients who excreted greater than or equal to 6 mg of bilirubin per day are alive (mean follow-up, 61 months) with normal or near-normal liver function. The severity of liver fibrosis, bile duct proliferation, and bile stasis did not correlate with survival, whereas giant cell transformation and parenchymal degeneration were highly significant (P less than or equal to .000 and P less than or equal to .0003, respectively). Twenty-six infants with grade 1-4 giant cell transformation or grade 2-4 parenchymal degeneration had a mean survival of 11 months.(ABSTRACT TRUNCATED AT 250 WORDS)