Effect of liver transplantation with primary hyperoxaluria type 1: Five case reports and review of literature.

Abstract

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 m2, 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 m2.

CONCLUSION:

Different transplantation strategies should be adopted for patients based on their renal function stage. Preemptive-LT offers a good therapeutic approach for PH1.

• Authors: Xin-Yue Wang, Zhi-Gui Zeng, Zhi-Jun Zhu, Lin Wei, Wei Qu, Ying Liu, Yu-Le Tan, Jun Wang, Hai-Ming Zhang, Wen Shi, Li-Ying Sun
• Journal: World journal of clinical cases (World J Clin Cases) Vol. 11 Issue 5 Pg. 1068-1076 (Feb 16 2023) ISSN: 2307-8960 [Print] United States
• PMID: 36874433 (Publication Type: Case Reports)
• Copyright: ©The Author(s) 2023. Published by Baishideng Publishing Group Inc. All rights reserved.