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

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.

Keywords: chronic renal failure; pediatric liver transplantation; primary hyperoxaluria type 2; simultaneous liver and kidney transplantation.

Figure 1

Showing, from pre-operative serum AST, ALT (Primary Axis), bilirubin Total, INR, Creatinine (Secondary Axis) till postoperative day (POD) 20. AST, Aspartate Amino transferase; ALT, Alanine Amino-transferase; INR, International Normalised Ratio.

Figure 2

Plain Abdominal CT (computed tomogram), showing nephrocalcinosis, with peritoneal dialysis catheter in situ. Boot-shaped heart can't be missed.

Figure 3

CT abdomen coronal section, showing bilateral nephrocalcinosis. CT, computed tomogram.

Figure 4

GRHPR enzyme in the cytosol of the hepatocyte, its role in glyoxalate metabolism. In the absence of GRHPR, lactate dehydrogenase (LDH) catalyzes the metabolism of hydroxypyruvate to l-glycerate and glyoxylate to oxalate, leading to oxalate accumulation in PH2. AGT, alanine glyoxylate aminotransferase; GRHPR, Glyoxylate reductase/hydroxypyruvate reductase.