10.6084/m9.figshare.5772657.v1 John Fredy Nieto Rios John Fredy Nieto Rios Monica Zuluaga Monica Zuluaga Lina Maria Serna Higuita Lina Maria Serna Higuita Adriana Florez Adriana Florez Diana Carolina Bello-Marquez Diana Carolina Bello-Marquez Arbey Aristizábal Arbey Aristizábal Catalina Ocampo Kohn Catalina Ocampo Kohn Gustavo Adolfo Zuluaga Gustavo Adolfo Zuluaga Primary hiperoxaluria diagnosed after kidney transplantation: report of 2 cases and literature review SciELO journals 2018 hyperoxaluria, primary kidney failure, chronic kidney transplantation lithiasis nephrocalcinosis 2018-01-10 06:01:37 Dataset https://scielo.figshare.com/articles/dataset/Primary_hiperoxaluria_diagnosed_after_kidney_transplantation_report_of_2_cases_and_literature_review/5772657 <div><p>Abstract Primary hyperoxaluria (PH) is a very rare genetic disorder; it is characterized by total or partial deficiency of the enzymes related to the metabolism of glyoxylate, with an overproduction of calcium oxalate that is deposited in different organs, mainly the kidney, leading to recurrent lithiasis, nephrocalcinosis and end stage renal disease (ESRD). In patients with ESRD that receive kidney transplantation alone, the disease has a relapse of 100%, with graft loss in a high percentage of patients in the first 5 years of transplantation. Three molecular disorders have been described in PH: mutation of the gene alanin glioxalate aminotransferase (AGXT); glyoxalate reductase/hydroxy pyruvate reductase (GRHPR) and 4-OH-2-oxoglutarate aldolase (HOGA1). We present two cases of patients with a history of renal lithiasis who were diagnosed with primary hyperoxaluria in the post-transplant period, manifested by early graft failure, with evidence of calcium oxalate crystals in renal biopsy, hyperoxaluria, hyperoxalemia, and genetic test compatible; they were managed with proper diet, abundant oral liquids, pyridoxine, hydrochlorothiazide and potassium citrate; however, they had slow but progressive deterioration of their grafts function until they reached end-stage chronic renal disease.</p></div>