title: Severe late acute allograft rejection in a child after living-related auxiliary partial orthotopic liver transplantation for ornithine transcarbamylase deficiency.
abstract: Auxiliary liver transplantation (ALT) is known to correct liver-based metabolic disorders. However, it remains unclear whether the presence of a native liver influences the long-term prognosis of ALT for metabolic diseases. We reported on a 4-yr-old girl who had undergone living-related auxiliary partial orthotopic liver transplantation (APOLT) for ornithine transcarbamylase deficiency and experienced severe late acute rejection 18 months after liver transplantation, during weaning of immunosuppressive agents. Results of histological analysis of the graft indicated very severe acute rejection (rejection activity index, 9/9), and computed tomography revealed graft liver atrophy. These observations suggest the possibility that severe rejection might occur in APOLT, especially during weaning of immunosuppression.
authors.affiliation: Department of Pediatrics, National Defense Medical College, Saitama, Tokorozawa City, Japan.
authors.forename: H
authors.initials: H
authors.lastname: Komatsu
authors.forename: A
authors.initials: A
authors.lastname: Inui
authors.forename: T
authors.initials: T
authors.lastname: Fujisawa
authors.forename: T
authors.initials: T
authors.lastname: Sogo
authors.forename: Y
authors.initials: Y
authors.lastname: Miyagawa
authors.forename: M
authors.initials: M
authors.lastname: Inui
authors.forename: S
authors.initials: S
authors.lastname: Uemoto
authors.forename: Y
authors.initials: Y
authors.lastname: Inomata
authors.forename: K
authors.initials: K
authors.lastname: Tanaka
mesh_terms: D000208:Acute Disease; D000592:Amino Acid Metabolism, Inborn Errors / Q000601:surgery*; D001706:Biopsy; D002675:Child, Preschool; D005260:Female; D006084:Graft Rejection / Q000175:diagnosis / Q000473:pathology; D006801:Humans; D008099:Liver / Q000473:pathology; D016031:Liver Transplantation; D019520:Living Donors; D020163:Ornithine Carbamoyltransferase Deficiency Disease