title: The first experience of sequential liver-kidney transplantation for the treatment of primary hyperoxaluria type-1 in Iran as a developing country.
abstract: Primary hyperoxaluria Type-1 (PH-1) is caused by a deficiency of alanine-glyoxylate aminotransferase manifesting as urolithiasis, nephrocalcinosis, and end-stage renal disease (ESRD). Among treatment options, best outcomes have been achieved by sequential liver-kidney transplantation (Seq-LKT). Herein, we report a patient with PH-1 and ESRD who underwent Seq-LKT in Iran. Criteria for diagnosis included a history of recurring calcium oxalate renal stones and elevated urine oxalate level combined with liver biopsy and absent enzymatic activity at the age of 13 years. Conservative treatment including pyridoxine, potassium citrate solution, high fluid intake, and hemodialysis was administered initially. Liver transplantation was performed at the age of 17 years from a deceased donor followed 4½ months later by a living-unrelated donor kidney transplantation. After two years of follow-up, the patient experienced no complications and had normal liver and renal function. This is the first successful experience of Seq-LKT in the treatment of PH-1 in Iran as a developing country with limited access to equipment and medications.
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.affiliation: Department of Kidney Transplantation, Dr. Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran.
authors.forename: Firouzeh
authors.initials: F
authors.lastname: Tabassomi
authors.affiliation: Department of Kidney Transplantation, Dr. Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran.
authors.forename: AmirHossein
authors.initials: A
authors.lastname: Latif
authors.affiliation: Department of Nephrology, Dr. Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran.
authors.forename: MohammadReza
authors.initials: M
authors.lastname: Ganji
mesh_terms: D003906:Developing Countries; D006801:Humans; D006959:Hyperoxaluria; D006960:Hyperoxaluria, Primary; D007492:Iran; D007676:Kidney Failure, Chronic; D016030:Kidney Transplantation; D008099:Liver; D016031:Liver Transplantation