Presentation of an acquired urea cycle disorder post liver transplantation

Marwan Ghabril, Justin Nguyen, David Kramer, Trina Genco, Martin Mai, Barry G. Rosser

Research output: Contribution to journalArticle

5 Citations (Scopus)

Abstract

The liver's role as the largest organ of metabolism and the unique and often critical function of liver-specific enzyme pathways imply a greater risk to the recipient of acquiring a donor metabolic disease with liver transplants versus other solid organ transplants. With clinical consequences rarely reported, the frequency of solid organ transplant transfer of metabolic disease is not known. Ornithine transcarbamylase deficiency (OTCD), although rare, is the most common of the urea cycle disorders (UCDs). Because of phenotypic heterogeneity, OTCD may go undiagnosed into adulthood. With over 5000 liver transplant procedures annually in the United States, the likelihood of unknowingly transmitting OTCD through liver transplantation is very low. We describe the clinical course of a liver transplant recipient presenting with acute hyperammonemia and encephalopathy after receiving a liver graft form a donor with unrecognized OTCD.

Original languageEnglish (US)
Pages (from-to)1714-1716
Number of pages3
JournalLiver Transplantation
Volume13
Issue number12
DOIs
StatePublished - Dec 2007
Externally publishedYes

Fingerprint

Inborn Urea Cycle Disorder
Ornithine Carbamoyltransferase Deficiency Disease
Liver Transplantation
Liver
Transplants
Metabolic Diseases
Tissue Donors
Hyperammonemia
Brain Diseases
Enzymes

ASJC Scopus subject areas

  • Surgery
  • Transplantation

Cite this

Ghabril, M., Nguyen, J., Kramer, D., Genco, T., Mai, M., & Rosser, B. G. (2007). Presentation of an acquired urea cycle disorder post liver transplantation. Liver Transplantation, 13(12), 1714-1716. https://doi.org/10.1002/lt.21291

Presentation of an acquired urea cycle disorder post liver transplantation. / Ghabril, Marwan; Nguyen, Justin; Kramer, David; Genco, Trina; Mai, Martin; Rosser, Barry G.

In: Liver Transplantation, Vol. 13, No. 12, 12.2007, p. 1714-1716.

Research output: Contribution to journalArticle

Ghabril, M, Nguyen, J, Kramer, D, Genco, T, Mai, M & Rosser, BG 2007, 'Presentation of an acquired urea cycle disorder post liver transplantation', Liver Transplantation, vol. 13, no. 12, pp. 1714-1716. https://doi.org/10.1002/lt.21291
Ghabril, Marwan ; Nguyen, Justin ; Kramer, David ; Genco, Trina ; Mai, Martin ; Rosser, Barry G. / Presentation of an acquired urea cycle disorder post liver transplantation. In: Liver Transplantation. 2007 ; Vol. 13, No. 12. pp. 1714-1716.
@article{507cb32fd96149949d9691d9c859c27b,
title = "Presentation of an acquired urea cycle disorder post liver transplantation",
abstract = "The liver's role as the largest organ of metabolism and the unique and often critical function of liver-specific enzyme pathways imply a greater risk to the recipient of acquiring a donor metabolic disease with liver transplants versus other solid organ transplants. With clinical consequences rarely reported, the frequency of solid organ transplant transfer of metabolic disease is not known. Ornithine transcarbamylase deficiency (OTCD), although rare, is the most common of the urea cycle disorders (UCDs). Because of phenotypic heterogeneity, OTCD may go undiagnosed into adulthood. With over 5000 liver transplant procedures annually in the United States, the likelihood of unknowingly transmitting OTCD through liver transplantation is very low. We describe the clinical course of a liver transplant recipient presenting with acute hyperammonemia and encephalopathy after receiving a liver graft form a donor with unrecognized OTCD.",
author = "Marwan Ghabril and Justin Nguyen and David Kramer and Trina Genco and Martin Mai and Rosser, {Barry G.}",
year = "2007",
month = "12",
doi = "10.1002/lt.21291",
language = "English (US)",
volume = "13",
pages = "1714--1716",
journal = "Liver Transplantation",
issn = "1527-6465",
publisher = "John Wiley and Sons Ltd",
number = "12",

}

TY - JOUR

T1 - Presentation of an acquired urea cycle disorder post liver transplantation

AU - Ghabril, Marwan

AU - Nguyen, Justin

AU - Kramer, David

AU - Genco, Trina

AU - Mai, Martin

AU - Rosser, Barry G.

PY - 2007/12

Y1 - 2007/12

N2 - The liver's role as the largest organ of metabolism and the unique and often critical function of liver-specific enzyme pathways imply a greater risk to the recipient of acquiring a donor metabolic disease with liver transplants versus other solid organ transplants. With clinical consequences rarely reported, the frequency of solid organ transplant transfer of metabolic disease is not known. Ornithine transcarbamylase deficiency (OTCD), although rare, is the most common of the urea cycle disorders (UCDs). Because of phenotypic heterogeneity, OTCD may go undiagnosed into adulthood. With over 5000 liver transplant procedures annually in the United States, the likelihood of unknowingly transmitting OTCD through liver transplantation is very low. We describe the clinical course of a liver transplant recipient presenting with acute hyperammonemia and encephalopathy after receiving a liver graft form a donor with unrecognized OTCD.

AB - The liver's role as the largest organ of metabolism and the unique and often critical function of liver-specific enzyme pathways imply a greater risk to the recipient of acquiring a donor metabolic disease with liver transplants versus other solid organ transplants. With clinical consequences rarely reported, the frequency of solid organ transplant transfer of metabolic disease is not known. Ornithine transcarbamylase deficiency (OTCD), although rare, is the most common of the urea cycle disorders (UCDs). Because of phenotypic heterogeneity, OTCD may go undiagnosed into adulthood. With over 5000 liver transplant procedures annually in the United States, the likelihood of unknowingly transmitting OTCD through liver transplantation is very low. We describe the clinical course of a liver transplant recipient presenting with acute hyperammonemia and encephalopathy after receiving a liver graft form a donor with unrecognized OTCD.

UR - http://www.scopus.com/inward/record.url?scp=37549002482&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=37549002482&partnerID=8YFLogxK

U2 - 10.1002/lt.21291

DO - 10.1002/lt.21291

M3 - Article

C2 - 18044746

AN - SCOPUS:37549002482

VL - 13

SP - 1714

EP - 1716

JO - Liver Transplantation

JF - Liver Transplantation

SN - 1527-6465

IS - 12

ER -