| Objective: Biliary atresia(BA) is one of the causes of obstructive jaundice in neonates. The histological appearance of the intra-and extrahepatic bile duct is characterized by progressive inflammation, leading to obliteration of intra-and extrahepatic bile duct, hepatic fibrosis, cholestatic cirrhosis and portal hypertension. Most of the patients would die before 2 years old without operation. Kasai operation -portoenterostomy has been accepted worldwide, for this procedure has improved the prognosis of the patients dramatically . The outcome of Kasai operation depends on the patient's age at operating , type of BA, severity of hepatic fibrosis and postoperative complications. Unfortunately, the long-term outcome of Kasai operation is not satisfactory. Survivors more than 10 years rang from 25% to 48%. It should be keeped in mind that BA is a kind of progressive disease. After successful Kasai operation , about 25% to 40%of BA patients have near-normal lives and are jaundice-free , but most of these patients develop progressive hepatic fibrosis and portal hypertension .Liver transplantation serves as a end-result in about 60%to 80% of patients with BA. Hepatic portoerterostomy wins time and enhances the chance of successful operation for the liver transplantation(LT).Besides surgical techniques, the prognosis of LT has a good correlation with the age of infants and general clinical status. So it is important to predict the prognosis of hepatic portoenterostomy in BA infants in the early stage of the disorder by biochemical , histochemical and immunohistochemical parameters. However, routine liver function indexes such as serum albumin, bilirubin, glutamic-pyruvic transaminase(GPT) are unable to predict the prognosis of BA clearly.It has been testified that mast cells (MC) take an important role in theprocess of liver fibrosis. The differatiation, development and activation of mast cells are dependent upon the stem cell factor(SCF)/c-kit ligand-receptor system. The c-kit expression in the liver consentrate mainly on the mast cells.The aim of the study is to search the relationship between the c-kit expression of the liver and the degree of liver fibrosis and the prognosis in BA.Methods: The imformation of BA infants at the Department of Pediatric Surgery of the Affiliated Hospital of Qingdao Medical College during 1996 to 2003 was collected. In this series, 20 patients' information is complete. Five liver samples of nomal infants serve as controls. The liver samples were taken from the margin of livers in the operations. Hepatic fibrosis is classified into I to IV grades according to Okumas' standards under HE- stains. Immunohistochemical PV 6000 two-step methods were taken respectively. Using a x20 objective c-kit positive cells were counted within 5 non-overlapping fields under microscope , and their average werecalculated, the data were expressed as mean ?standard error( X 眘).Mean serum value of total albumin, bilirubin, direct bilirubin and GPT pre-operation --portoenterostomy in BA were summarized and calculated retrospectively .Results: Patients were divided into two groups(good and poor outcome groups) according to their outcome of hepatic portoenteros- tomy.All indicators were analyzed between the two groups. Prognosis of BA is correlated with the age (days) at operation(good outcome group 59.80?1.50, poor outcome group 103.67?8.60, p<0.05), without serum value of total albumin, bilirubin, direct bilirubin, GPT and the degree of liver fibrosis.C-kit positive cells are mostly discovered in peri-portal tract and rarely in fibroconnective tissues. The c-kit+ cells are spindle-shaped , brown-yellow coloured and with roughly granular cytoplasma.The number of c-kit+ cells in BA patients was higher than in controls(BA 19.37?.39 ,controls 1.8?.86,p< 0.001); higher in poor outcome group than in good outcome group ( 21.03?.80,vs14.40?.93, p< 0.05); higher in more severe fibrosis group than in more mild fibrosis group ( 24.7?.17,vs 14.04?.38,p< 0.001)... |
PDF Full Text Request