Orthotopic Liver Transplantation for Mitochondrial Respiratory Chain Disorders: A Study of Five Children

During the last two decades, mitochondrial respiratory chain disorders have been recognized to cause acute and chronic liver failure, usually associated with variable involvement of other organs (1). Over the same period of time, liver transplantation has become an accepted treatment option for end-stage liver disease, with only a few definite contraindications, such as extrahepatic disseminated malignancy or multiorgan failure. The number of patients awaiting liver transplantation is on a continuous increase, despite attempts at enlarging the donor pool by surgical innovations including living related donation. It is therefore important to have a judicious approach to transplantation for conditions where the long-term outcome is poor or unknown. These would include mitochondrial respiratory chain disorders. In this issue of Transplantation Dubern et al.(2) report their experience of orthotopic liver transplantation in five children affected by this condition. The outcome is variable toward survival and progression of the disease in other organs, but of the three survivors, only one appears to have no obvious extrahepatic involvement.