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Liver transplantation in hemophilia A

Bontempo, FA and Lewis, JH and Gorenc, TJ and Spero, JA and Ragni, MV and Scott, JP and Starzl, TE (1987) Liver transplantation in hemophilia A. Blood, 69 (6). 1721 - 1724. ISSN 0006-4971

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Abstract

Four patients with hemophilia A have undergone liver transplantation in our institution, three successfully. The first was a 21-year-old man with chronic active hepatitis (CAH) in whom the effects of previous abdominal operations prevented the satisfactory technical insertion of the new liver. He died intraoperatively. The second patient was a 15-year-old boy with CAH who began to synthesize factor VIII coagulant activity (F VIII:C) within 18 hours of successful liver transplantation and has continued to do so for almost 2 years (F VIII:C range 0.89 to 3.20 U/mL). The first 2 months of his postoperative course were complicated by infections, but since that time he has done well and has returned to school. The third patient was a 48-year-old man with portal fibrosis and severe ascites. He synthesized F VIII:C (range 0.96 to 1.50 U/mL) within six hours after reestablishment of circulation through the new liver. His postoperative course was complicated by numerous infections, and he died with sepsis and an acquired immunodeficiency-like syndrome 4 months after transplantation. The fourth patient was a 47-year-old mild hemophiliac with CAH who produced adequate factor VIII:C levels following transplantation (range 0.79 to 2.80 U/mL). These patients demonstrate that liver transplantation in hemophiliacs with end-stage liver disease may be lifesaving and results in correction of the F VIII:C deficiency and associated hemorrhagic tendency.


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Details

Item Type: Article
Status: Published
Creators/Authors:
CreatorsEmailPitt UsernameORCID
Bontempo, FA
Lewis, JH
Gorenc, TJ
Spero, JA
Ragni, MV
Scott, JP
Starzl, TEtes11@pitt.eduTES11
Centers: Other Centers, Institutes, or Units > Thomas E. Starzl Transplantation Institute
Date: 21 August 1987
Date Type: Publication
Journal or Publication Title: Blood
Volume: 69
Number: 6
Page Range: 1721 - 1724
Institution: University of Pittsburgh
Refereed: Yes
ISSN: 0006-4971
Other ID: uls-drl:31735062119007, Starzl CV No. 706
Date Deposited: 08 Apr 2010 17:12
Last Modified: 05 Feb 2019 14:55
URI: http://d-scholarship.pitt.edu/id/eprint/4092

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