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The Capital Region of Denmark - a part of Copenhagen University Hospital
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First example of hepatocyte transplantation to alleviate ornithine transcarbamylase deficiency, monitored by NMR-based metabonomics

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  • Cristina Legido-Quigley
  • Olivier Cloarec
  • David A Parker
  • Gerard M Murphy
  • Elaine Holmes
  • John C Lindon
  • Jeremy K Nicholson
  • Ragai R Mitry
  • Hector Vilca-Melendez
  • Mohamed Rela
  • Anil Dhawan
  • Nigel Heaton
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We demonstrate the effective use of NMR spectroscopic profiles of urine and plasma from the first successful use of hepatocyte transplantation as a bridge to auxiliary partial orthotopic liver transplantation in a child antenatally diagnosed with severe ornithine transcarbamylase deficiency. In this single-patient study, NMR profiles indicated that the disrupted urea cycle could be normalized by hepatocyte cell infusion and this was confirmed using orthogonal partial least-squares-based chemometrics. However, despite dietary manipulations and adminstration of ammonia scavengers, the desired reduction in plasma ammonia was not consistently achieved between sessions of hepatocyte transplantation due to episodes of sepsis. A subsequent liver transplant corrected the metabolic abnormalities. The use of metabolic profiling has been shown to be a promising method for evaluating the efficacy of cell infusions and has demonstrated the capability for the early detection of response to therapy in real time, an approach that may be of use in wider clinical settings.

Original languageEnglish
JournalBioanalysis
Volume1
Issue number9
Pages (from-to)1527-35
Number of pages9
ISSN1757-6180
DOIs
Publication statusPublished - Dec 2009
Externally publishedYes

    Research areas

  • Ammonia/blood, Hepatocytes/physiology, Humans, Infant, Infant, Newborn, Liver Transplantation, Magnetic Resonance Spectroscopy, Male, Metabolomics/methods, Monitoring, Physiologic/methods, Mutation, Ornithine Carbamoyltransferase Deficiency Disease/blood, Sepsis/blood, Treatment Outcome, Urea/blood

ID: 54961634