Successful kidney transplantation from a brain-dead donor with ornithine transcarbamylase deficiency

Ornithine transcarbamylase (OTC) deficiency is the most common inherited urea cycle disorder (1). Occasionally, the initial symptoms of this disease present in a late-onset form and may cause severe hyperammonemia with cerebral edema and brain death. We present a 44-year-old male patient who died of cerebral edema due to severe hyperammonemia secondary to OTC deficiency. The two kidneys were successfully transplanted in two patients. After a follow-up period of 2 years, both recipients were in stable clinical condition and graft function was good.