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Effect of liver transplantation with primary hyperoxaluria type 1: Five case reports and review of literature
BACKGROUND: Primary hyperoxaluria type 1 (PH1) is a rare autosomal recessive disease stemming from a deficiency in liver-specific alanine-glyoxylate aminotransferase, resulting in increased endogenous oxalate deposition and end-stage renal disease. Organ transplantation is the only effective treatme...
Autores principales: | , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Baishideng Publishing Group Inc
2023
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9979304/ https://www.ncbi.nlm.nih.gov/pubmed/36874433 http://dx.doi.org/10.12998/wjcc.v11.i5.1068 |
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author | Wang, Xin-Yue Zeng, Zhi-Gui Zhu, Zhi-Jun Wei, Lin Qu, Wei Liu, Ying Tan, Yu-Le Wang, Jun Zhang, Hai-Ming Shi, Wen Sun, Li-Ying |
author_facet | Wang, Xin-Yue Zeng, Zhi-Gui Zhu, Zhi-Jun Wei, Lin Qu, Wei Liu, Ying Tan, Yu-Le Wang, Jun Zhang, Hai-Ming Shi, Wen Sun, Li-Ying |
author_sort | Wang, Xin-Yue |
collection | PubMed |
description | BACKGROUND: Primary hyperoxaluria type 1 (PH1) is a rare autosomal recessive disease stemming from a deficiency in liver-specific alanine-glyoxylate aminotransferase, resulting in increased endogenous oxalate deposition and end-stage renal disease. Organ transplantation is the only effective treatment. However, its approach and timing remain controversial. CASE SUMMARY: We retrospectively analyzed 5 patients diagnosed with PH1 from the Liver Transplant Center of the Beijing Friendship Hospital from March 2017 to December 2020. Our cohort included 4 males and 1 female. The median age at onset was 4.0 years (range: 1.0-5.0), age at diagnosis was 12.2 years (range: 6.7-23.5), age at liver transplantation (LT) was 12.2 years (range: 7.0-25.1), and the follow-up time was 26.3 mo (range: 12.8-40.1). All patients had delayed diagnosis, and 3 patients had progressed to end-stage renal disease by the time they were diagnosed. Two patients received preemptive LT; their estimated glomerular filtration rate was maintained at > 120 mL/min/1.73 m(2), indicating a better prognosis. Three patients received sequential liver and kidney transplantation. After transplantation, serum and urinary oxalate decreased, and liver function recovered. At the last follow-up, the estimated glomerular filtration rates of the latter 3 patients were 179, 52 and 21 mL/min/1.73 m(2). CONCLUSION: Different transplantation strategies should be adopted for patients based on their renal function stage. Preemptive-LT offers a good therapeutic approach for PH1. |
format | Online Article Text |
id | pubmed-9979304 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | Baishideng Publishing Group Inc |
record_format | MEDLINE/PubMed |
spelling | pubmed-99793042023-03-03 Effect of liver transplantation with primary hyperoxaluria type 1: Five case reports and review of literature Wang, Xin-Yue Zeng, Zhi-Gui Zhu, Zhi-Jun Wei, Lin Qu, Wei Liu, Ying Tan, Yu-Le Wang, Jun Zhang, Hai-Ming Shi, Wen Sun, Li-Ying World J Clin Cases Case Report BACKGROUND: Primary hyperoxaluria type 1 (PH1) is a rare autosomal recessive disease stemming from a deficiency in liver-specific alanine-glyoxylate aminotransferase, resulting in increased endogenous oxalate deposition and end-stage renal disease. Organ transplantation is the only effective treatment. However, its approach and timing remain controversial. CASE SUMMARY: We retrospectively analyzed 5 patients diagnosed with PH1 from the Liver Transplant Center of the Beijing Friendship Hospital from March 2017 to December 2020. Our cohort included 4 males and 1 female. The median age at onset was 4.0 years (range: 1.0-5.0), age at diagnosis was 12.2 years (range: 6.7-23.5), age at liver transplantation (LT) was 12.2 years (range: 7.0-25.1), and the follow-up time was 26.3 mo (range: 12.8-40.1). All patients had delayed diagnosis, and 3 patients had progressed to end-stage renal disease by the time they were diagnosed. Two patients received preemptive LT; their estimated glomerular filtration rate was maintained at > 120 mL/min/1.73 m(2), indicating a better prognosis. Three patients received sequential liver and kidney transplantation. After transplantation, serum and urinary oxalate decreased, and liver function recovered. At the last follow-up, the estimated glomerular filtration rates of the latter 3 patients were 179, 52 and 21 mL/min/1.73 m(2). CONCLUSION: Different transplantation strategies should be adopted for patients based on their renal function stage. Preemptive-LT offers a good therapeutic approach for PH1. Baishideng Publishing Group Inc 2023-02-16 2023-02-16 /pmc/articles/PMC9979304/ /pubmed/36874433 http://dx.doi.org/10.12998/wjcc.v11.i5.1068 Text en ©The Author(s) 2023. Published by Baishideng Publishing Group Inc. All rights reserved. https://creativecommons.org/licenses/by-nc/4.0/This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. |
spellingShingle | Case Report Wang, Xin-Yue Zeng, Zhi-Gui Zhu, Zhi-Jun Wei, Lin Qu, Wei Liu, Ying Tan, Yu-Le Wang, Jun Zhang, Hai-Ming Shi, Wen Sun, Li-Ying Effect of liver transplantation with primary hyperoxaluria type 1: Five case reports and review of literature |
title | Effect of liver transplantation with primary hyperoxaluria type 1: Five case reports and review of literature |
title_full | Effect of liver transplantation with primary hyperoxaluria type 1: Five case reports and review of literature |
title_fullStr | Effect of liver transplantation with primary hyperoxaluria type 1: Five case reports and review of literature |
title_full_unstemmed | Effect of liver transplantation with primary hyperoxaluria type 1: Five case reports and review of literature |
title_short | Effect of liver transplantation with primary hyperoxaluria type 1: Five case reports and review of literature |
title_sort | effect of liver transplantation with primary hyperoxaluria type 1: five case reports and review of literature |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9979304/ https://www.ncbi.nlm.nih.gov/pubmed/36874433 http://dx.doi.org/10.12998/wjcc.v11.i5.1068 |
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