Primary hyperoxaluria type II and organ transplantation
10.3969/j.issn.1674-7445.2023145
- VernacularTitle:原发性高草酸尿症II型与器官移植
- Author:
Yiling FANG
1
;
Yun MIAO
1
Author Information
1. Department of Organ Transplantation, Nanfang Hospital of Southern Medical University, Guangzhou 510515, China.
- Publication Type:TransplantationForefront
- Keywords:
Primary hyperoxaluria type Ⅱ;
Kidney stone;
End-stage renal disease (ESRD);
Kidney transplantation;
Combined liver and kidney transplantation;
Calcium oxalate stone
- From:
Organ Transplantation
2023;14(6):804-809
- CountryChina
- Language:Chinese
-
Abstract:
Primary hyperoxaluria type Ⅱ (PH2) is an inherited disorder of the glyoxylate metabolism caused by the gene mutation of glyoxylate reductase/hydroxypyruvate reductase (GRHPR). PH2 is characterized by recurrent nephrolithiasis and nephrocalcinosis, which may even progress into end-stage renal disease. Currently, organ transplantation is the only treatment option for PH2, which mainly includes two strategies: kidney transplantation and combined liver and kidney transplantation. Kidney transplantation yields a high risk of recurrence of oxalate nephropathy, which may cause early graft dysfunction. Combined liver and kidney transplantation could mitigate the deficiency of oxalate metabolism, whereas it yields a high risk of graft complications. PH2 is an extremely rare disorder. No consensus has been reached on the indications, surgical selection and perioperative management of organ transplantation for PH2 patients. In this article, the pathogenesis, diagnosis, monitoring and organ transplantation experience of PH2 were reviewed, aiming to divert clinicians' attention to PH2 and provide reference for determining diagnosis and treatment regimens, especially transplantation strategy for PH2 patients.