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Liver transplantation for arteriohepatic dysplasia (Alagille's syndrome)

Marino, IR and ChapChap, P and Esquivel, CO and Zetti, G and Carone, E and Borland, L and Tzakis, AG and Todo, S and Rowe, MI and Starzl, TE (1992) Liver transplantation for arteriohepatic dysplasia (Alagille's syndrome). Transplant International, 5 (2). 61 - 64. ISSN 0934-0874

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Thirteen out of 268 children (<18 years old) underwent hepatic transplantation (OLT) for end-stage liver disease (ESLD) associated with arteriohepatic dysplasia (AHD). Seven children are alive and well with normal liver function. Six children died, four within 11 days of the operation and the other two at 4 and 10 months after the OLT. Vascular complications with associated septicemia were responsible for the deaths of three children. Two died of heart failure and circulatory collapse, secondary to pulmonary hypertension and congenital heart disease. The remaining patient died of overwhelming sepsis not associated with technical complications. Seven patients had a portoenterostomy or portocholecystostomy early in life; five of these died after the OLT. Severe cardiovascular abnormalities in some of our patients suggest that complete hemodynamic monitoring with invasive studies should be performed in all patients with AHD, especially in cases of documented hypertrophy of the right ventricle. The improved quality of life in our surviving patients confirms the validity of OLT as a treatment of choice in cases of ESLD due to AHD. © 1992 Springer-Verlag.


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Item Type: Article
Status: Published
CreatorsEmailPitt UsernameORCID
Marino, IR
ChapChap, P
Esquivel, CO
Zetti, G
Carone, E
Borland, L
Tzakis, AG
Todo, S
Rowe, MI
Starzl, TEtes11@pitt.eduTES11
Centers: Other Centers, Institutes, Offices, or Units > Thomas E. Starzl Transplantation Institute
Date: 1 May 1992
Date Type: Publication
Journal or Publication Title: Transplant International
Volume: 5
Number: 2
Page Range: 61 - 64
DOI or Unique Handle: 10.1007/bf00339217
Institution: University of Pittsburgh
Refereed: Yes
ISSN: 0934-0874
Other ID: uls-drl:31735062113281, Starzl CV No. 1535
Date Deposited: 08 Apr 2010 17:26
Last Modified: 02 Feb 2019 13:56


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