Medknow Publications and Staff Society of Seth GS Medical College and KEM Hospital, Mumbai, India
Abstract
Primary hyperoxaluria is a rare autosomal recessive disease due to
deficiency of an oxalate-metabolizing liver enzyme, which results in
nephrolithiasis and renal failure. Concomitant liver and kidney
transplant is recommended as isolated kidney transplant is inevitably
complicated by recurrence of the disease. We present a 25-year-old man
with end-stage nephrolithiatic renal disease who underwent bilateral
nephrectomy, followed by kidney transplantation. There was progressive
worsening of kidney function two weeks post transplant. Review of
nephrectomy and transplant kidney biopsy showed abundant calcium
oxalate crystals and further workup revealed hyperoxaluria, which was
previously unsuspected. Later he developed fever, breathlessness,
hemiparesis and died 10 weeks after transplant. Autopsy revealed
multi-organ deposits of oxalate crystals as well as widespread
zygomycosis. This case emphasizes the need for careful pre-transplant
evaluation of patients with renal calculus disease in order to exclude
primary hyperoxaluria