Glycogenosis storage type I diseases and evolutive adenomatosis: an indication for liver transplantation.

Details

Serval ID
serval:BIB_37FA4482CA60
Type
Article: article from journal or magazin.
Publication sub-type
Case report (case report): feedback on an observation with a short commentary.
Collection
Publications
Title
Glycogenosis storage type I diseases and evolutive adenomatosis: an indication for liver transplantation.
Journal
Transplant International : Official Journal of the European Society For Organ Transplantation
Author(s)
Lerut J.P., Ciccarelli O., Sempoux C., Danse E., deFlandre J., Horsmans Y., Sokal E., Otte J.B.
ISSN
0934-0874 (Print)
ISSN-L
0934-0874
Publication state
Published
Issued date
2003
Peer-reviewed
Oui
Volume
16
Number
12
Pages
879-884
Language
english
Notes
Publication types: Case Reports ; Journal ArticlePublication Status: ppublish
Abstract
We report on two cases of type I glycogen storage disease (GSD) complicated by malignant tumors. A 23-year-old man had GSD Ia with adenomatosis. He underwent transplantation for rapidly growing and radiologically changing adenomata. At histological examination, one adenoma had become a hepatocellular carcinoma. A 22-year-old, HBV-infected woman had GSD type Ib with adenomatosis. At follow-up, several tumors showed changing morphological characteristics. Pre-transplant laparotomy confirmed the presence of a metastatic cholangiocarcinoma. Liver transplantation should be considered in GSD type I patients with adenomatosis, especially when tumor characteristics change. Regular detailed Doppler ultrasound and magnetic nuclear resonance screening during childhood and adolescence are, therefore, mandatory in order for the timing of transplantation to be optimized.
Keywords
Adenoma, Liver Cell/complications, Adenoma, Liver Cell/pathology, Adult, Cholangiocarcinoma/complications, Cholangiocarcinoma/pathology, Fatal Outcome, Female, Glycogen Storage Disease Type I/complications, Humans, Liver Neoplasms/complications, Liver Neoplasms/pathology, Liver Transplantation, Male
Pubmed
Web of science
Create date
20/10/2016 17:25
Last modification date
20/08/2019 14:26
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