Histopathologic Perspective of Combined Liver-kidney Transplant: In Primary Hyperoxaluria Type 1 Patient

Primary hyperoxaluria (PH) type 1 is a rare autosomal recessive disorder of glyoxylate metabolism. Its prevalence is 1-3 cases/million people. Glyoxylate is the precursor of oxalate which is believed to be produced by oxidation in liver peroxisomes. Serine-pyruvate aminotransferase/alanine-glyoxylate aminotransferase is an enzyme involved in the metabolism of glyoxylate. In the absence of this enzyme, oxalate and glycolate are overproduced leading to hyperoxaluria. This causes urolithiasis or nephrocalcinosis, which are conditions caused by the deposition of calcium oxalate. Due to its rarity and heterogeneous phenotype, it remains unrecognized due to which diagnosis is delayed, ending up in end-stage renal disease (ESRD) and ultimately death. Hence, early diagnosis and simultaneous hepatorenal transplant remain the mainstay to avoid systemic oxalosis. Here, we discuss a case of a 43-year-old female who underwent combined liver-kidney transplant with a history of multiple episodes of renal calculi since childhood ultimately landing into ESRD in view of PH type 1.