|| Checking for direct PDF access through Ovid
Primary oxaloses are rare genetic disorders that result in hepatic overproduction of oxalate. Primary hyperoxaluria type1(PH1) is the most common and severe form. Clinical manifestations range from renal calculi to progressive renal impairment and systemic deposition of oxalate. Supportive measures may slow kidney disease progression but many cases develop end stage renal disease(ESRD). Combined liver-kidney transplantation may be curative.A 65yo male had developed ESRD in 1990 and underwent deceased-donor kidney transplantation in Holland in 2000(?). The transplant failed within a week, histology revealed oxalate crystals and a diagnosis of PH1 was made by genetic analysis revealing compound pyridoxine-sensitive mutations in the AGXT gene. The patient returned to haemodialysis for 8 years then presented to our unit for simultaneous kidney-liver transplantation(SLK). Aiming to prevent early disease recurrence, Continuous Renal Replacement Therapy(CRRT) was performed during surgery followed by daily CRRT for nine days. Liver allograft function was achieved, however the kidney experienced delayed graft function. A renal biopsy showed only acute tubular necrosis and intermittent haemodialysis was continued for a further week by which time kidney function was evident. The patient was discharged on day 26, creatinine 178umol/L. On day 29, creatinine was 202umol/L and a renal biopsy revealed focal oxalate crystals affecting 10% of kidney. A Repeat biopsy at day 39 showed progressive oxalate deposition. Daily dialysis was re-established followed by slow recovery of kidney allograft function.Data surrounding transplantation for primary hyperoxyluria are limited to small case series. Risk of disease recurrence caused by renal excretion of residual systemic oxalate may occur despite SLK preventing further production. More data on how to assess oxalate burden and prevent recurrence are required.