Open AccessThe first experience of sequential liver-kidney transplantation for the treatment of primary hyperoxaluria type-1 in Iran as a developing country
Author(s) -
Gh. Naderi,
Firouzeh Tabassomi,
AmirHossein Latif,
M.R Ganji
Publication year - 2016
Publication title -
saudi journal of kidney diseases and transplantation/našrat amraḍ wa zira'aẗ al-kulaẗ
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.268
H-Index - 30
eISSN - 2320-3838
pISSN - 1319-2442
DOI - 10.4103/1319-2442.185262
Subject(s) - primary hyperoxaluria , medicine , nephrocalcinosis , transplantation , liver transplantation , kidney transplantation , hemodialysis , gastroenterology , kidney , renal function , urology , surgery
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.