Biliary atresis

Organ transplantation has moved over 30 years from being experimental and heroic to being the treatment of choice in many terminal diseases of vital organs, such as biliary atresis, which would require a liver transplant, or pulmonary fibrosis, which would require a heart/lung transplant. There are now many more older and chronically sick people than ever before (Hudak et al, 1998). Transplantation offers hope for an improved quality of life. All patients have a right to care, although there remains a dichotomy between the holistic model of care and the medical model...

It is well known that prognosis is very poor in patients with severe hepatic insufficiency such as congenital biliary atresia and fulminant hepatitis. The liver transplantation is only effective therapy for these patients and living-related liver transplantation is becoming popular in Japan. We observed the changes in serum human hepatocyte growth factor (h-HGF) levels of the recipients during the operation in 3 cases of congenital atresis and one of fulminant hepatitis. Serum h-HGF values in these patients reached the maximal levels (5-10-fold compared to the base line values) at the phase of portal or hepatic artery anastomosis during the operation...

Early surgical intervention in cases of extrahepatic biliary atresia improves prognosis. The ratio of serum gamma-glutamyl transpeptidase to SGOT is elevated in infants with infantile obstructive cholangiopathy. This appears to be a sensitive method for distinguishing infants with extrahepatic biliary atresis from those with neonatal hepatitis. This distinction was evident as early as 5 to 14 days of age and was clearly manifest in ten of 12 infants with biliary atresia. The ratio was also elevated in patients with alpha 1-antitrypsin deficiency who had bile duct proliferation...

Between March 1963 and January 1978, 74 patients 18 years of age or younger have had liver replacements at the University of Colorado Medical Center, Denver. The most common cause of native liver failure was biliary atresis (48/74, 65%); the second most common cause was chronic aggressive hepatitis (12/74, 16%). Twenty-nine patients (39%) lived for at least one year, and 16 are still alive one to nine years after transplantation. Technical surgical problems, rejection, and infection were the main causes of death...