Document 1250
 DOCN  M9591250
 TI    Orthotopic liver transplantation in a patient with severe hemophilia A:
       a life-saving treatment for the first Italian case.
 DT    9509
 AU    Federici AB; Mannucci PM; Stabile F; Rossi G; Piseddu G; Angelo Bianchi
       Bonomi Hemophilia and Thrombosis Center, Institute; of Internal
       Medicine, Milan, Italy.
 SO    Int J Clin Lab Res. 1995;25(1):44-6. Unique Identifier : AIDSLINE
       MED/95306816
 AB    Clinical cure of hemophilia A by orthotopic liver transplantation has
       been reported in 11 cases. We describe the first successful Italian
       case. A 27-year-old man had cirrhosis caused by previous infections with
       the hepatitis B, C and D viruses following life-long treatment with
       factor VIII concentrates made from large plasma pools. He was, however,
       seronegative for the human immunodeficiency virus. In the year before
       transplantation, life-threatening gastrointestinal bleeding due to
       severe esophageal varices required a large transfusion regimen (on
       average, 13 bags of red cell concentrates and 35,000 U of factor
       VIII/week). To perform orthotopic liver transplantation 8,000 U of
       factor VIII were given during surgery together with 10 bags of red cells
       and 11 of fresh-frozen plasma. Intraoperative bleeding was not different
       from that of non-hemophilic patients undergoing orthotopic liver
       transplantation. No additional factor VIII was used after
       transplantation and factor VIII levels in plasma were always above 50
       U/dl, reaching the highest value of 184 U/dl on day 4 post
       transplantation. He was discharged from hospital 10 weeks after
       transplantation with factor VIII levels of 68 U/dl. All virological
       markers are currently negative, except anti-hepatitis C virus
       antibodies. In this patient orthotopic liver transplantation was a
       life-saving treatment for end-stage cirrhosis and a cure for hemophilia
       A.
 DE    von Willebrand Factor/THERAPEUTIC USE  Adult  Case Report  Factor
       VIII/THERAPEUTIC USE  Hemophilia/*SURGERY  Human  Italy  Liver
       Cirrhosis/SURGERY  Liver Failure/SURGERY  *Liver Transplantation  Male
       JOURNAL ARTICLE

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