J.Jpn. Surg. Soc.. 97(8): 657-662, 1996

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Feature topic

LIVER TRANSPLANTATION AS A THERAPEUTIC MODALITY OF BILIARY ATRESIA

One hundred fifty eight patients with bilairy atresia (BA) have undergone living related liver transplantation (LRLT) in our institution since June 1990. Since the start of our LRLT program we have not experienced any death of BA patients who were initially treated in our hospital. All the 158 patients have been transplanted after Kasai operation. Re-do Kasai after failed cases should be very carefully indicated because of potentially bad effect on the following transplantation. Poor body weight gain is the first sign of liver failure in infants, and indicates the timing for LRLT. In older children, there are many kinds of indications, including itching of skin, frequent abscence of the school due to admission, and intrapulmonary shunt. One third of the transplanted patients necessitated LRLT before 1 year old. LRLT in infantile period is the first step to save all patients with BA. Technically, portal vein plasty and enterolysis are the key factors in transplants for BA patients. Main cause of death after transplantation was infection. Bacterial culuture at the porta hepatis is imporant to know the pathogen in the posttransplant infection. Posttransplant bone-densitmetry disclosed that physical growth is enhanced after 3 months, when steroid is weaned off. For the best quality of life, first Kasai alld following LRLT is going to be a widely accepted therapeutic modality for BA patients.

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