Abstract
Purpose
To investigate the efficacy of living donor liver transplantation (LDLT) plus domino-auxiliary partial orthotopic liver transplantation (D-APOLT) in pediatric patients with metabolic disorders.
Methods
From May 2017 to October 2018, two patients with ornithine aminotransferase deficiency (OTCD) and one patient with type I Crigler-Najjar syndrome (CNS1) received LDLT, their livers were prepared as donors for D-APOLT. Two patients with CNS1 received domino liver grafts from OTCD patients; one OTCD patient received a domino liver graft from a CNS1 patient.
Results
The mean follow-up was 26.6 months. The liver function and ammonia remained in the normal range at the end of the follow-up in all recipients. One D-APOLT patient experienced portal vein thrombosis 2 days after transplantation and required reoperation, this patient presented an imbalance of portal blood perfusion between the native and the domino liver at 8 months after liver transplant. The imbalance was improved by interventional radiology treatment. Two LDLT patients experienced early mild acute rejection.
Conclusions
The non-cirrhotic livers from pediatric patients with metabolic liver disease can be used as domino donor grafts for selected pediatric patients with different metabolic liver disease. D-APOLT achieves ideal recipient outcomes and provides a strategy to expand donor source for children.
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Abbreviations
- ALT:
-
Alanine transaminase
- AST:
-
Aspartate transaminase
- APOLT:
-
Auxiliary partial orthotopic liver transplantation
- CNS1:
-
Crigler-Najjar syndrome
- CT:
-
Computed tomography
- D-APOLT:
-
Domino-auxiliary partial orthotopic liver transplantation
- HV:
-
Hepatic vein
- LDLT:
-
Living donor liver transplantation
- LLS:
-
Left lateral segment
- LT:
-
Liver transplantation
- MHV:
-
Middle hepatic vein
- MLD:
-
Metabolic liver diseases
- MRCP:
-
Magnetic resonance cholangiopancreatography
- NCMLD:
-
Non-cirrhotic metabolic liver diseases
- OTCD:
-
Ornithine aminotransferase deficiency
- PLT:
-
Partial liver transplantation
- PV:
-
Portal vein
- RL:
-
Right lobe
- TB:
-
Total bilirubin
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Funding
This study was funded by Tianjin Clinical Research Center for Organ Transplantation Project (15ZXLCSY00070).
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All the procedures in studies involving human participants were in accordance with the institutional review board of Tianjin First Center Hospital (approval number: 2018N148KY) and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.
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Dong, C., Song, Z., Meng, X. et al. Successful living donor liver transplantation plus domino-auxiliary partial orthotopic liver transplantation for pediatric patients with metabolic disorders. Pediatr Surg Int 36, 1443–1450 (2020). https://doi.org/10.1007/s00383-020-04756-3
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DOI: https://doi.org/10.1007/s00383-020-04756-3