Mitochondrial respiratory chain hepatopathies: Role of liver transplantation. A case series of five patients

Abstract

Introduction: Orthotopic liver transplantation (OLT) in patients with mitochondrial respiratory chain disorders (MRCD) is controversial because of possible multi-organ involvement. Aim: To illustrate the clinical diversity of MRCD, the difficulty in making an accurate tissue diagnosis and whether to undertake OLT in five patients with proven MRCD. A review of the reported cases in the literature is presented. Methods: Retrospective chart review from 1995 to 2007 at a paediatric liver transplant centre where five children with hepatic MRCD were identified. Results: Patient 1 was transplanted for ‘cryptogenic’ cirrhosis. The diagnosis of MRCD was made on the explant. The patient remains well 5..