Regressive course of oxalate deposition in primary hyperoxaluria after kidney transplantation

Abstract

Primary hyperoxaluria (PH) is a rare autosomal recessive disease caused by the functional defect of alanine-glyoxylate aminotransferase (AGT) enzyme in the liver and it is characterized by the deposition of diffuse calcium oxalate crystals. A 38-year-old male patient presented with history of recurrent nephrolithiasis and has received chronic hemodialysis treatment for 2 years. Cadaveric renal transplantation was applied to the case. The patient was reoperated on postoperative day 13 because of the collection surrounding the urethra. During this operation, kidney biopsy was made due to late decrease in creatinine levels. Deposition of diffuse oxalate crystal was detected in allograft kidney biopsy, whereas in the 0-hour biopsy there were no oxalate crystals. Oxalate level was found to be high in a 24-hour urine specimen (118 mg/L, normal level: 7-44 mg/L). The patient was identified with primary hyperoxaluria and followed up in terms of systemic oxalate deposition as well as allograft kidney. In the kidney biopsy taken after 18 months, we detected that oxalate crystals almost entirely disappeared. In our case, bilateral preretinal, intraretinal, and intravascular diffuse oxalate crystals were detected, and argon laser photocoagulation treatments were needed for choroidal and retinal neovascularization. Repeated ophthalmic examinations showed the regressive nature of oxalate depositions. In the 18th month, fundus examination and fluorescein angiography revealed that oxalate crystals were significantly regressed. To increase the quality of life and slow down the systemic effects of oxalosis, kidney-only transplantation is beneficial.

Oxalate retinopathy is irreversible despite early combined liver-kidney transplantation in primary hyperoxaluria type 1

“…The severity of the retinal manifestation and subsequent visual impairment is associated primarily with renal function and is not based on specific genotype‐phenotype correlations. Single case reports demonstrate stable or partially reversal neuroretinal oxalate depositions in response to renal or combined hepatorenal transplantation, yet to the best of our knowledge, no larger transplanted IO cohort has been analyzed longitudinally based on their ocular findings.…”

“…Celik et al described a case of a 38‐year‐old male patient with regressive ROx after renal transplantation; yet visual acuity is not shown . In a 59‐year‐old woman with PH1, pyridoxine supplementation, low oxalate diet, and an increased frequency of dialysis after renal transplantation appeared to result in decreased ROx …”

Massive bone marrow involvement in an end stage renal failure case with erythropoietin-resistant anemia and primary hyperoxaluria

“…16 The transplantation options given to PH1 cases include isolated kidney, isolated liver and combined kidney and liver transplantations. 4 Isolated kidney transplantation would result in significant decrease in oxalate levels however, due to the continued excessive production of oxalate; ESRD will quickly develop in the transplanted kidney. 17 Isolated liver transplantation is only implemented prior to the development of any kidney damage.…”

“…17 Isolated liver transplantation is only implemented prior to the development of any kidney damage. 4 The other transplantation option for PH1 cases is the combined kidney-liver transplantation and is the most common cause of kidney-liver transplantations in literature. 17 The transplantation procedure especially for infants and cases of ESRD are first liver then kidney transplantations.…”

“…1 Primary hyperoxaluria type 1 (PH1) occurs due to the functional defect of the liver enzyme alanine aminotransferase (AGT). [2][3][4] Primary hyperoxaluria type 2 (PH2) is rare and develops secondary to failure of the cytosolic enzyme D-glycerate dehydrogenase. 3 Both primary hyperoxaluria types result in excessive production of oxalate and are clinically characterized by hyperoxaluria.…”