Abstract
Background: It is to research the validity of medical strategy and the importance of excluding biliary atresia (BA) from metabolic cholestasis in premature infant. Methods: We investigated retrospectively the clinical data for two premature neonates; one was an extremely low birthweight infant with BA (case 1) and the other was a very low birthweight infant with metabolic disorder of bile acid (case 2). We analyzed the justification of the clinical strategy and the medical management through various tests. Among the recipients who underwent pediatric living donor liver transplantation (LDLT) at Kyoto University Hospital starting in June 1990, the number of the recipients less than 5kg in body weight at pediatric LDLT was 20 cases (5 boys: 15 girls). We evaluated the survival rate, the morbidity, and the mortality in the recipients. Results: Case 1 died due to multiple organ failure on the 36th postoperative day. Case 2 takes further investigative studies and medical management. Of 20 recipients, 10 survived and 10 died. All five recipients less than 3.7kg in body weight at pediatric LDLT died, and the survival rate of the recipients more than 3.8kg in body weight was 66.7%. Conclusion: It is important for the premature infants of metabolic cholestasis to rule out BA by means of ultrasound study or radioisotope scintigraphy. Pediatric LDLT should be taken into consideration in case of the optimal chance of Kasai's procedure being missed or progressing unknown liver cirrhosis.
