Journal
JOURNAL OF HEPATOLOGY
Volume 51, Issue 3, Pages 483-490Publisher
ELSEVIER
DOI: 10.1016/j.jhep.2009.05.026
Keywords
Glycogen storage disease Ia; Liver transplantation; Hepatocellular adenoma; Hepatocellular carcinoma
Categories
Ask authors/readers for more resources
Background/Aims: Hepatocellular carcinoma (HCC) most often occurs within hepatocellular adenomas (HCAs) in glycogen storage disease Ia (GSD Ia) patients. The objective of this retrospective study is to assess outcomes after liver transplantation (LT) for GSD la where the principal indication for transplantation was prevention of HCC. Methods: Petitions to the United Network for Organ Sharing region 11 review board for additional model for end-stage liver disease listing points were made on behalf of GSD la patients. Demographics, pre-operative comorbidity, and outcomes for GSD Ia patients who underwent LT were reviewed. Results: Between 2004 and 2006, five GSD Ia patients underwent LT. Multiple HCAs with focal hemorrhage and/or necrosis but without histological evidence of malignancy were identified in all explanted specimens. Four of five patients had complications after LT, including cytomegalovirus (CMV) infections and steroid responsive allograft rejection. Hemoglobin levels and serum triglyceride, total cholesterol, blood glucose, and lactic acid concentrations improved in all patients after LT. Corn starch feeding was not required in any patient after LT. Renal function worsened in three patients despite modifications to primary immunosuppressive medications. All patients are alive at last follow-up (range 25-48 months) and all post-transplant complications have resolved. Conclusions: By removing all possible adenomatous tissue and reversing the underlying hepatic enzymatic deficiency LT provides definitive prevention against HCC and correction of most metabolic derangements in GSD Ia patients. Renal dysfunction secondary to GSD Ia persists-underscoring the need for further studies to better understand the mechanisms of renal dysfunction in these patients. (C) 2009 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved.
Authors
I am an author on this paper
Click your name to claim this paper and add it to your profile.
Reviews
Recommended
No Data Available