The usefulness of immunohistochemical staining of bile tracts in biliary atresia.

AIM OF THE STUDY: To assess ductular proliferation (DP) and ductal plate malformation (DPM) in biliary atresia (BA) by means of immunohistochemical staining using cytokeratins CK7 and CK19 and neural cell adhesion molecule (NCAM) antibody CD56. MATERIAL AND METHODS: In 10 cases of BA, liver surgical biopsies obtained at the time of hepatoportoenterostomy were stained with H&E, PAS, Gomori and Azan methods. Immunohistochemical technique was used to outline bile ducts, ductular reaction, reactive bile duct/ductules and DPM by CK7, CK19 and NCAM antibody CD56. RESULTS: We found fibrosis, bile stasis and mild inflammation in all cases. In the routine staining DP was not seen in 3 cases. The immunohistochemical staining by means of CK19 was helpful in the detection of DP, and allowed it to be demonstrated in all cases. The biliary epithelial cell markers for CD56, CK7, CK19 were used for demonstration of bile duct cell but not hepatocyte alterations in the structure of intrahepatic biliary ducts and different stages of maturation. CD56 as a marker of immature bile ducts was expressed on biliary epithelium of bile ducts and bizarre forms of DPM in 6 cases. The positive expression of CD56 corresponded to the co-localization of CK19 of DPM, but not CK7, to the ductular reaction at the limiting plate of portal tracts. CD7, considered as a marker of DP, also stained ductal hepatocytes and multipotential oval cells, and was a marker of DPM in 3 cases. CONCLUSIONS: Use of CK7, CK19 and CD56 is helpful in BA diagnosis and allows differentiation of the stage of developing bile duct cells according to the expression pattern.

Liver transplantation in children with biliary atresia and polysplenia syndrome.

BACKGROUND: Children with biliary atresia and polysplenia syndrome (BA-PS) have always been considered as high risk liver transplant recipients due to technical problems during transplant surgery. We report single-center experience with liver transplantation in children with this syndrome. MATERIAL/METHODS: Between 2000 and 2010, 401 liver transplantations were performed in 358 children, including 6 patients with BA-PS, who underwent living (5 patients) or deceased (1 patient) donor liver transplantation. Patients demonstrated various malformations: absence of retrohepatic vena cava (3), intestinal malrotation (3), preduodenal portal vein (1), hepatic artery anomalies (3), cardiac anomalies (2), and situs inversus (1). Transplantations were performed at the patient age of 8 months to 11 years. RESULTS: There were no serious technical problems during the operations, and we did not have to use vascular conduits for graft revascularization in any case. All patents were alive at follow-up between 14 and 123 months after transplantation (mean 75 months). We observed, however, increased incidence of PV thrombosis and biliary complications in these patients, which did not influence patient and graft survival. In 1 child with graft failure due to chronic rejection after discontinuation of immunosuppression due to PTLD, retransplantation was performed. CONCLUSIONS: Results of liver transplantation in children with BA-PS are as good as for other indications and non-syndromic BA in an experienced pediatric liver transplant center. Although there were no serious technical problems during deceased or living related donor transplantation in these children, close observation for possible vascular complications should be the routine in the postoperative period.

Liver transplantation with ABO incompatible graft under immunoadsorption protocol--case report.

BACKGROUND: ABO incompatible liver transplantation is still controversial, but accepted in selected cases. Recently several authors reported use of the new technology aimed at elimination anti-donor ABO specific hemagglutinins to assist immunosuppression in preventing acute rejection after transplantation. CASE REPORT: We report two cases of liver transplantation in children with ABO incompatible graft under immunoadsorption protocol. Both patients were transplanted urgently (one due to acute decompensation of chronic liver failure and second due to acute liver failure) with ABO incompatible liver grafts. Both patients were in very poor general condition with deterioration of neurological status and there were no suitable ABO compatible grafts at the time. In both cases immunosuppressive protocol consisted of induction with basiliximab, followed by tacrolimus, mycophenolate mofetil and corticosteroids. Additionally in both patients 3 immunoadsorption sessions with Glycosorb ABO® system (Glycorex AB, Sweden), were performed. There were no any acute rejection episodes till now. The only problem observed after transplantation was mild anemia due to low grade hemolysis in the postoperative period. Both patients are alive and well with very good liver function 20 and 26 months after transplantation. CONCLUSIONS: Immunoadsorption therapy can be safely and effectively introduced in recipients of ABO incompatible donor liver.

Hemodynamic failure as an indication to urgent liver transplantation in infants with giant hepatic hemangiomas or vascular malformations--report of four cases.

The aim of this study was to present acute hemodynamic failure as a rare indication for liver transplantation in neonates and infants with liver hemangiomatosis. We report four patients aged one to six months with giant liver hemangiomas, with huge arterio-venous shunting within these malformations. In three, many skin hemangiomas were found. All children developed right ventricular failure. In two, a trial of pharmacological reduction was attempted with corticosteroids and cyclophosphamide. In one patient, the arterio-venous fistulas were embolized without any improvement in hemodynamic status. Two children underwent rescue hepatic artery surgical ligation, which did not prevent heart and then multiorgan failure including liver failure. After unsuccessful conventional therapy, all infants were considered for urgent liver transplantation; in three cases, it was performed with a living-related donor, and in one case with a deceased donor. All patients are alive and well with the follow-up between nine and 37 months after transplantation. Liver transplantation should be considered as a rescue treatment in children with hepatic vascular malformations leading to hemodynamic insufficiency when conventional therapy is unsuccessful and multiorgan failure develops.