Late diagnosis of hyperoxaluria in a patient after kidney transplantation – case report
Journal Title: Postępy Nauk Medycznych - Year 2014, Vol 27, Issue 2
Abstract
Primary hyperoxaluria (PH) is a rare genetic disorder that leads to elevated excretion of calcium oxalates and its deposition in tissues. First symptoms usually occur under the age of five and consist of massive parenchymal oxalosis or bilateral renal calculi. Clinical manifestations in later stages of disease, when the glomerular filtration decreases, can consist of cardiac conduction defects, distal gangrene, dificulties with vascular access, synovitis. We present a case of a 65-year old female patient referred to the Department of Transplantation Medicine and Nephrology 22 days after kidney transplantation with end stage renal disease (ESRD) after a right-sided nephrectomy due to pyonephrosis caused by incidental unilateral kidney calculi with a history of recurrent urinary tract infections. After receiving a kidney transplantation the patient developed delayed graft function with preserved urine output. Kidney biopsies revealed an increasing amount of tubular calcium oxalate depositions and persistent acute tubular necrosis. Calcium oxalate excretion was insignificantly elevated. Ultrasound scans revealed presence of transplanted kidney nephrolithiasis. We raised a suspicion of PH. We started a 4-weeks course of daily high-flux hemodialysis and also treated the patient with pyridoxamine and citric acid combined with potassium citrate and sodium citrate. Control kidney biopsy 2 months after the transplantation revealed absence of calcium oxalate depositions with present unresolved ATN and moderate de novo atherosclerosis. Therapy proved to be unsuccessful and the patient returned to the haemodialysis program. The diagnosis of PH was later confirmed by the presence of a typical C508G > a mutation. Based on this case we conclude that a late onset of PH with non-specific symptoms is possible. Guidelines for the exclusion of PH in adult patients with ESRD of unknown origin are needed, especially before kidney transplantation.
Authors and Affiliations
Maciej Sawosz, Tomasz Cieciura, Agnieszka Perkowska-Ptasińska, Przemysław Sikora, Bodo Beck, Andrzej Chmura, Magdalena Durlik
Keywords
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