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.