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Liver-Kidney Transplantation in Primary Hyperoxaluria Type-1: Case Report and Literature Review
Primary hyperoxaluria type-1 (PH1) is a rare inherited autosomal recessive disorder in which a deficiency of the hepatic enzyme alanine-glyoxylate aminotransferase leads to endogenous oxalate overproduction, renal failure, systemic oxalate deposition and death. As hemodialysis provides insufficient...
Autores principales: | , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Avicenna Organ Transplantation Institute
2011
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4089259/ https://www.ncbi.nlm.nih.gov/pubmed/25013605 |
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author | Siegal, D. Su, W. S. DaBreo, D. Puglia, M. Gregor, L. Gangji, A. S. |
author_facet | Siegal, D. Su, W. S. DaBreo, D. Puglia, M. Gregor, L. Gangji, A. S. |
author_sort | Siegal, D. |
collection | PubMed |
description | Primary hyperoxaluria type-1 (PH1) is a rare inherited autosomal recessive disorder in which a deficiency of the hepatic enzyme alanine-glyoxylate aminotransferase leads to endogenous oxalate overproduction, renal failure, systemic oxalate deposition and death. As hemodialysis provides insufficient oxalate clearance, patients ultimately require both liver and kidney transplantation for correction of the metabolic abnormality and oxalate excretion. Herein, we describe a young adult male with end-stage renal disease and systemic oxalosis causing progressive disabling multi-organ dysfunction while awaiting transplantation. We review the literature regarding liver-kidney transplantation and suggest that for patients with PH1, a standardized assessment of organ dysfunction and functional impairment may improve identification of patients requiring urgent transplantation thereby reducing the morbidity and mortality that can occur with delayed transplantation. |
format | Online Article Text |
id | pubmed-4089259 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2011 |
publisher | Avicenna Organ Transplantation Institute |
record_format | MEDLINE/PubMed |
spelling | pubmed-40892592014-07-10 Liver-Kidney Transplantation in Primary Hyperoxaluria Type-1: Case Report and Literature Review Siegal, D. Su, W. S. DaBreo, D. Puglia, M. Gregor, L. Gangji, A. S. Int J Organ Transplant Med Case Report Primary hyperoxaluria type-1 (PH1) is a rare inherited autosomal recessive disorder in which a deficiency of the hepatic enzyme alanine-glyoxylate aminotransferase leads to endogenous oxalate overproduction, renal failure, systemic oxalate deposition and death. As hemodialysis provides insufficient oxalate clearance, patients ultimately require both liver and kidney transplantation for correction of the metabolic abnormality and oxalate excretion. Herein, we describe a young adult male with end-stage renal disease and systemic oxalosis causing progressive disabling multi-organ dysfunction while awaiting transplantation. We review the literature regarding liver-kidney transplantation and suggest that for patients with PH1, a standardized assessment of organ dysfunction and functional impairment may improve identification of patients requiring urgent transplantation thereby reducing the morbidity and mortality that can occur with delayed transplantation. Avicenna Organ Transplantation Institute 2011 2011-08-01 /pmc/articles/PMC4089259/ /pubmed/25013605 Text en This is an Open Access article distributed under the terms of the Creative Commons Attribution License, (http://creativecommons.org/licenses/by/3.0/) which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Case Report Siegal, D. Su, W. S. DaBreo, D. Puglia, M. Gregor, L. Gangji, A. S. Liver-Kidney Transplantation in Primary Hyperoxaluria Type-1: Case Report and Literature Review |
title | Liver-Kidney Transplantation in Primary Hyperoxaluria Type-1: Case Report and Literature Review |
title_full | Liver-Kidney Transplantation in Primary Hyperoxaluria Type-1: Case Report and Literature Review |
title_fullStr | Liver-Kidney Transplantation in Primary Hyperoxaluria Type-1: Case Report and Literature Review |
title_full_unstemmed | Liver-Kidney Transplantation in Primary Hyperoxaluria Type-1: Case Report and Literature Review |
title_short | Liver-Kidney Transplantation in Primary Hyperoxaluria Type-1: Case Report and Literature Review |
title_sort | liver-kidney transplantation in primary hyperoxaluria type-1: case report and literature review |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4089259/ https://www.ncbi.nlm.nih.gov/pubmed/25013605 |
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