PMID: 7546154Jan 1, 1995Paper

Heterozygous protein C deficiency and dysfibrinogenemia acquired by liver transplantation

Transplant International : Official Journal of the European Society for Organ Transplantation
M CransacJ Saric

Abstract

Orthotopic liver transplantation is now a successful treatment for end-stage liver diseases. Since most components of the coagulation system are synthesized by liver parenchymal cells, there is always a risk of genetic defects of hemostasis being transmitting by liver transplantation. Some coagulation factor defects, such as protein C deficiency, do not induce abnormalities in routine coagulation tests and, thus, go undetected before organ procurement. We report the first case, to our knowledge, of the transmission of heterozygous protein C deficiency, an autosomal recessive genetic defect, associated with dysfibrinogenemia, an autosomal dominant trait, by liver transplantation. Both the recipient and the donor presented with severe thrombotic complications. This case shows that potentially morbid genetic defects can be transmitted by organ transplantation, and it emphasizes the difficulty associated with organ procurement criteria, particularly for liver transplantation, in which routine blood tests appear insufficient for determining whether or not organs can or should be procured from a given donor.

References

Dec 1, 1991·Annals of Internal Medicine·K ClarksonW Bell
Sep 1, 1990·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·R L StahlW D Warren
Oct 12, 1989·The New England Journal of Medicine·T E StarzlD Van Thiel
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Oct 15, 1987·The New England Journal of Medicine·J MiletichG Broze
Nov 1, 1993·Scandinavian Journal of Clinical and Laboratory Investigation·J V SørensenF Knudsen

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Citations

Aug 26, 1999·Transplant International : Official Journal of the European Society for Organ Transplantation·S R GuyC M Miller
Oct 28, 1998·The Journal of Clinical Investigation·L R JalbertF J Castellino
Jul 11, 2006·American Journal of Transplantation : Official Journal of the American Society of Transplantation and the American Society of Transplant Surgeons·N K OsbornK V Narayanan Menon

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