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.

Preclinical experiment of auxiliary partial orthotopic liver transplantation as a curative treatment for hemophilia

“…Recently, auxiliary partial orthotopic liver transplantation (APOLT) has been indicated for patients with acute liver failure, considering the possibility of recovery of the patient's own liver 11–13. APOLT has also been performed for limited number of patients with metabolic liver diseases 14–17. Because the liver function of patients with metabolic diseases remains normal except for production of the deficient metabolic factor, the patient survival is secured by preserved native liver even in case of graft loss.…”

Antibodies against cytokeratin 8/18 in a patient with de novo autoimmune hepatitis after living-donor liver transplantation

“…One patient (patient 8) had an episode of ACR 3 months after LDLT, and 4 patients (patients 1, 2, 3, 6) each had 1 episode of late ACR at 17 months, 9 months, 18 months, and 31 months after transplantation, respectively. Patient 6, who was receiving auxiliary partial orthotopic liver transplantation, had a severe late ACR from which she was rescued by ligation of the right portal branch of the native liver 13. Those 5 patients (patients 1, 2, 3, 6, 8) and the remaining patients (patients 4 and 5) who experienced no episodes of ACR or late ACR were also seronegative for all the examined autoantibodies during the follow‐up period.…”

Interdisziplinäre Behandlung des frühmanifesten Ornithintranscarbamylase(OTC)-Mangels1 - Zwei Patientenberichte und Literaturübersicht -

“…Bei Patienten mit Harnstoffzyklusstörungen, die sich weniger gut durch eine konservative medikamentöse Therapie behandeln lassen als unser Patient 2, kann alternativ eine Lebertransplantation erwogen werden [24,25,26]. Erfolgt diese vor der Manifestation neurologischer Defizite, ist eine normale intellektuelle Entwicklung möglich [27].…”