Maggio 2002 - Volume XXI - numero 5
Focus
Dipartimento di Medicina della Procreazione e dell’Età Evolutiva, Scuola di Specialità in Pediatria, Università di Pisa
Key words: Autoimmune hepatitis, Interface hepatitis, Immunosuppressive therapy, Cyclosporine, Liver transplantation
Autoimmune hepatitis (AIH) is an uncommon disease occurring mainly in women and characterised by the morphological changes of interface hepatitis on liver biopsy, hypergammaglobulinemia, elevated serum aminotransferases, and circulating autoantibodies. AIH is thought to have a basis in aberrant autoreactivity to hepatocyte antigens in genetically predisposed individuals. AIH is notably heterogeneous with respect to its clinical expression and laboratory features and patients may present without obvious clinical evidence of liver disease or with an acute hepatitis. Corticosteroids alone or in conjunction with azathioprine are the treatment of choice and result in remission induction in over 90% of patients. Sustained response to therapy may result in substantial regression of fibrosis even in advanced cases. Rapid withdrawal of immunosuppression is associated with high risk of disease relapse in many patients. Alternative strategies in patients who have failed to achieve remission on “standard therapy” include the use of cyclosporine. Liver transplantation is the treatment of choice in managing patients with decompensated disease unresponsive to “rescue” medical treatment.
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Focus
U.O. Pediatria, U.O. Chirurgia III e Centro Trapianto di Fegato, Ospedali Riuniti di Bergamo
Key words: Liver transplantation, Biliary tree atresia, Tacrolimus
The Authors report their experience on liver transplantation in children at the Pediatric Liver Transplantation Program, Ospedali Riuniti of Bergamo. Over a five year period 156 transplantations have been performed in 141 children (15 children required retransplantation). Most interventions have been performed by the “split in situ” technique. The main indication (62% of all cases) was biliary tree atresia. The median age was 1,5 years. The average waiting time to surgery was less than one month. Vascular complications occurred in 14 cases; biliary complications in 25 cases. Since tacrolimus has been used for immunosuppression, the likelihood of acute rejection has been lowered to less than 20%. Free yearsurvival its over 85%.
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Focus
Gastroenterologia ed Epatologia Pediatrica, I Divisione di Pediatria, Dipartimento di Medicina della Procreazione e dell’Età Evolutiva, Università di Pisa
Key words: Cholestasis, Epstein-Barr virus (EBV), Infectious mononucleosis
Although biochemical evidence of liver damage is frequent in children with EBV infectious mononucleosis, signs or symptoms related to an impaired bile flow as jaundice or pruritus are rare. The Authors report three cases observed in a two-year period with acute EBV infectious mononucleosis presenting with cholestasis and abrupt onset, and describe findings of a retrospective review for evidence of liver involvement of 54 children with EBV related infectious mononucleosis. Biochemical evidence of liver damage was present in 27 patients with two patterns: isolated increase of aminotransferase with normal serum γ-glutamyl transpeptidase (γGT) activity in 16, and simultaneous increase of aminotransferase and γGT activity in 10. All patients recovered within a period of 2 weeks to 3 months. EBV infection may start with cholestasis with a biochemical pattern characterised by a significant increase of γGT, but a moderate increase of aminotransferase. Elevation of γGT in EBV infectious mononucleosis suggests a virus-induced, self-limited cholangiocyte damage.
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