Simultaneous Liver Kidney Transplantation in a Primary Type 2 Hyperoxaluria With Corrected TOF and Severe Cardiomyopathy: A Case Report

IF 3.3 Q2 GASTROENTEROLOGY & HEPATOLOGY Journal of Clinical and Experimental Hepatology Pub Date : 2024-04-16 DOI:10.1016/j.jceh.2024.101425

Navaneethan Subramanian , Abhishek Yadav , Jithin S. Kumar , George P. Abraham

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Abstract

Background

Primary type 2 hyperoxaluria is a very rare genetic disorder,^1,2 where in the progression to renal failure was assumed to be insidious and not very common.³ PH2 is due to deficient glyoxylate reductase/hydroxypyruvate reductase (GRHPR),^1,2 which was thought to have extra-hepatic production also.⁴ The progression to renal failure in these patient subgroups is well documented in the Literature and the role of SLK (simultaneous liver and kidney transplantation) has not been clearly established.⁸

Method

We present a case report of a young girl with PH2, who successfully underwent SLK, with evidence of reduction in the urine oxalate levels post SLK.

Results

PH2, though a rare genetic disease, has a proven potential to progress to chronic renal failure requiring transplantation, renal transplantation alone has not shown any benefit, these patients can be offered SLK as a primary treatment option, to improve the outcomes, this needs further validation with consensus and studies.

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同时进行肝肾移植治疗原发性2型高草酸尿症伴矫正性TOF和严重心肌病：病例报告。

背景原发性2型高草酸尿症是一种非常罕见的遗传性疾病，1,2 其中肾功能衰竭的进展被认为是隐匿的，并不常见。这些亚组患者发展为肾衰竭的情况在文献中已有详细记载，而 SLK（同时进行肝脏和肾脏移植）的作用尚未明确确定。结果PH2 虽然是一种罕见的遗传性疾病，但已被证实有可能发展为慢性肾功能衰竭，需要进行移植手术，而单独进行肾移植手术并未显示出任何益处，这些患者可选择 SLK 作为主要治疗方案，以改善预后，但这需要进一步的共识和研究来验证。

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