Outcomes of Living Kidney Transplantation for Mitochondrial Disease Patients: A Case Series.

• We evaluated the outcomes of 4 kidney transplant recipients with mitochondrial disease. • None of the recipients experienced rejection. • The grafted kidney of the patient who received it from her mother lost function at 5 years post-transplantation due to focal segmental glomerular sclerosis. • A patient with mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes died 2 years after transplantation due to encephalopathy progression with stable kidney function. Mitochondrial disease can affect many organs, including the brain, nerves, heart, liver, eyes, ears, pancreas, and kidneys. Kidney transplantation is a treatment option for renal failure due to mitochondrial disease; however, the prognosis of patients who undergo kidney transplantation for mitochondrial disease is unknown. Here we evaluated the outcomes of kidney transplant recipients with mitochondrial disease. Clinical data were obtained from 4 kidney transplantation recipients who were followed at our department. Of the 4 transplantations, 3 were performed in our department: 2 patients received kidneys from their fathers, and a third from his wife. The fourth recipient received a kidney from her mother—who had a mitochondrial genetic abnormality—at another hospital. Of the 4 recipients, 3 were diagnosed with mitochondrial disease before the transplantation, and the fourth was diagnosed after. All recipients had sensorineural deafness and diabetes mellitus, and only 1 had a history of encephalopathy and stroke-like episodes before the transplantation. One patient died 2 years after transplantation due to encephalopathy progression with stable kidney function. The grafted kidney of the patient who received it from her mother lost function at 5 years post-transplantation. A graft biopsy revealed focal segmental glomerular sclerosis due to mitochondrial disease. The other patients' kidney functions remained stable. None of the recipients experienced rejection. In kidney transplantation for mitochondrial disease, attention should be paid to the exacerbation of comorbidities, while careful consideration should be given to donors with a mitochondrial genetic abnormality. [ABSTRACT FROM AUTHOR]