Introduction:
Renal transplantation is the treatment of choice for end stage renal disease. It improves the quality of life and improves the survival . There could be numerous roadblocks to a good graft outcome . It could be immunological and non immunological. Understanding the native kidney disease is a crucial point in successful transplant. We describe the case of a 47 year old female who presented to us with end stage renal disease, underwent live renal transplant and attained a normal nadir creatinine after which the true disease unfolded before us to take away the graft.
Methods:
The 47 year old female was detected to have elevated creatinine 1 year prior to the presentation which rapidly progressed to end stage renal disease and was initiated on dialysis . Ultrasound of the native kidney showed bilateral small sized kidneys. she gave history of passing a single stone 10 years back and never had any more episodes of stone disease. Her urine was inactive and had no proteinuria. All the subsequent ultrasound of the native kidneys were normal. Patient was presumed to have chronic interstitial nephritis. Renal biopsy could not be done in view of bilateral small sized kidneys . Patient was worked up for renal transplant with her friend as the donor. Patient attained a nadir creatinine of 0.9 mg/ dl was discharged home. Patient developed graft dysfunction on post operative day 30 . Graft doppler showed parvus tardus wave form . She was taken up for angiogram and angioplasty with stenting was done.Patients’ renal functions improved mildly post stenting but continued to worsen subsequently . A graft biopsy was done which showed acute cellular rejection (i2, t3) with oxalate crystals seen in less than 5 % tubules . She was treated with injection methyl prednisolone followed by high dose prednisolone. Her renal functions continued to worsen . In the back ground of oxalate crystals in the tubules in the graft biopsy and oxalate crystals in urine examination , past history of stone disease , persistent graft dysfunction, she was suspected with hyperoxaluria . .
Results:
An X ray of kidney , urinary bladder showed bilateral nephrocalcinosis , Computerised tomography of abdomen showed cortical and medullary nephrocalcinosis and multiple calculi in the transplant kidney and ureter. 24 hour urinary oxalate levels were 80 mg. Genetic analysis was sent and the patient was reported to have AGXT mutation which was homozygous and pathogenic. Patient was diagnosed with primary hyperoxaluria with recurrence in the graft kidney with graft dysfunction. She was started on supportive therapy with high dose pyridoxine and oxalobacter fomigenes . Patient continued to have worsening renal function and became dialysis dependent at the end of 3 months post renal transplant. she has been registered for combined liver kidney transplant.
Conclusions:
Primary hyperoxaluria is an autosomal recessive disorder with variable presentation from being asymptomatic through mild stone disease to severe renal failure. The absence of severe stone disease with repeated normal ultrasounds of the native kidneys , absence of family history of kidney disease or stones, history of a single calculi about 10 years back made us overlook the possibility of hyperoxaluria in our patient. A computerised tomography of the kidney urinary bladder in patients with stone disease would help to identify such patients when the ultrasound might miss the possibility of nephrocalcinosis. We should have a low threshold for genetic studies in patients where the native kidney disease is not fairly identified from history , clinical examination and basic laboratory evaluation. Diseases which might look benign in the form of an interstitial disease might also recur in the graft kidney as in the case of primary hyperoxaluria
I have no potential conflict of interest to disclose.
I did not use generative AI and AI-assisted technologies in the writing process.