title: Failure of isolated kidney transplantation in a pediatric patient with primary hyperoxaluria type 2.
abstract: PH type 2 is caused by decreased activity of GRHPR enzyme that eventually leads to ESRD and systemic oxalosis. Here, we describe an Iranian pediatric patient with PH2 and early ESRD development who received recommended treatment by undergoing isolated kidney transplantation. Diagnosis criteria included a history of reoccurring calcium oxalate renal stones and elevated oxalate levels combined with liver biopsy and decreased enzymatic activity at age five. ESRD prompted transplantation and was performed at age nine. On Day 12 post-op, his serum creatinine level increased. A graft biopsy showed calcium oxalate crystal deposits in renal tubes with no evidence of acute rejection, which resolved with intensive hydration and administration of a potassium citrate solution. Subsequent biopsies confirmed results found in first biopsy. Despite the immunosuppressive therapy, his serum creatinine level increased again after 11 months. Renal tubular obstruction then led to graft nephrectomy. Pathological analysis of tissue confirmed findings of past biopsies. This was a very rare case of early ESRD in PH2 resulting in a failed isolated kidney transplant. As the GRHPR enzyme is predominantly expressed in liver, we suggest a combined liver-kidney transplant may be beneficial in patients with PH2.
authors.affiliation: Department of Kidney Transplantation, Dr. Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran.
authors.forename: GholamHossein
authors.initials: G
authors.lastname: Naderi
authors.forename: AmirHossein
authors.initials: A
authors.lastname: Latif
authors.forename: Firouzeh
authors.initials: F
authors.lastname: Tabassomi
authors.forename: Seyed Taher
authors.initials: ST
authors.lastname: Esfahani
mesh_terms: D000429:Alcohol Oxidoreductases / Q000378:metabolism; D001706:Biopsy; D002129:Calcium Oxalate / Q000737:chemistry; D002648:Child; D003404:Creatinine / Q000097:blood; D006084:Graft Rejection; D006801:Humans; D006960:Hyperoxaluria, Primary / Q000150:complications / Q000628:therapy*; D007492:Iran; D007669:Kidney Calculi / Q000150:complications*; D007676:Kidney Failure, Chronic / Q000150:complications / Q000628:therapy*; D016030:Kidney Transplantation; D008099:Liver / Q000201:enzymology; D017093:Liver Failure; D008297:Male; D009392:Nephrectomy; D016896:Treatment Outcome
chemical_list: D002129:Calcium Oxalate; D003404:Creatinine; D000429:Alcohol Oxidoreductases; C020830:glyoxylate reductase
keywords: Iran; graft loss; kidney transplantation; oxalosis; primary hyperoxaluria type 2; treatment