Date: Sunday, April 30, 2017
Session Name: Poster Session B: Pediatric Liver Transplant - Clinical
Session Time: 6:00pm-7:00pm
Presentation Time: 6:00pm-7:00pm
Location: Hall D1
The importance of ascertaining the cause of brain death in the donor is vital . We present a case of cadaveric liver donation (seg 2 and 3) to a paediatric patient who had previous chronic rejection of liver transplant for fulminant non A-G hepatitis.
It was later found out that the donor had OTC (Ornithine Transcarbomoylase Deficiency). The issue came in to focus when the adult recipient of the split liver developed encephalopathy and died. Genetic testing of the donor liver revealed OTC gene mutation. The death of the donor was due to seizures and encephalopathy. After further testing of donor body fluids and tissue it was identified that the donor died due to seizures and encephalopathy resulting from an undiagnosed urea cycle disorder (OTC deficiency)
The paediatric recipient with the OTC deficient liver is managed under the guidance of the metabolic team with restriction of protein intake and diet supplementation at times of illness or stress. The patient is doing well.
The lesson that we learnt was education and awareness of OTC deficiency in adult patients and the importance of having a clear cause of brain death in the donor.
CITATION INFORMATION: George M, Thomas G, Shun A, Stormon M. Ornithine Transcarbamylase Deficiency (OTC) in the Donor Liver, the Importance of Ascertaining the Cause of Death in the Brain Dead Donor. Am J Transplant. 2017;17 (suppl 3).
To cite this abstract in AMA style:George M, Thomas G, Shun A, Stormon M. Ornithine Transcarbamylase Deficiency (OTC) in the Donor Liver, the Importance of Ascertaining the Cause of Death in the Brain Dead Donor. [abstract]. Am J Transplant. 2017; 17 (suppl 3). https://atcmeetingabstracts.com/abstract/ornithine-transcarbamylase-deficiency-otc-in-the-donor-liver-the-importance-of-ascertaining-the-cause-of-death-in-the-brain-dead-donor/. Accessed November 26, 2020.
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