1. Exchange of Partial Liver Transplantation Between Children with Different Non-Cirrhotic Metabolic Liver Diseases: How Do We Arrive There?
- Author
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Cheng-Yen Chen, Hsin-Lin Tsai, Che-Chuan Loong, Cheng-Yuan Hsia, Chinsu Liu, and Niang-Cheng Lin
- Subjects
Liver surgery ,medicine.medical_specialty ,Pathology ,Enzyme deficiency ,Orthotopic liver transplantation ,medicine.medical_treatment ,Liver transplantation ,Gastroenterology ,03 medical and health sciences ,0302 clinical medicine ,030225 pediatrics ,Internal medicine ,Medicine ,Humans ,Child ,Transplantation ,business.industry ,Liver Diseases ,Patient Selection ,General Medicine ,Organ Size ,Enzymes ,Liver Transplantation ,Liver graft ,surgical procedures, operative ,Liver ,030220 oncology & carcinogenesis ,Liver functions ,business ,Liver pathology ,Metabolism, Inborn Errors - Abstract
Hepatic-based metabolic disorders are characterized by an enzyme deficiency expressed solely or mainly in the liver. They are divided into cirrhotic or non-cirrhotic metabolic liver diseases (NCMLDs), and most of them can be treated by liver transplantation. Because the livers with NCMLDs are usually structurally and functionally normal, the primary aim of the liver graft is to support the deficient enzymes rather than maintaining liver functions. Hence, we hypothesize that the exchange of partial liver grafts by the technique of auxiliary partial orthotopic liver transplantation (APOLT) between patients with 2 different NCMLDs may be feasible to replace the deficient enzymes in each patient. This hypothesis is based on the following conditions: (i) the patients have no chance of undergoing timely liver transplantation, (ii) the symptoms of each NCMLD may be alleviated after exchanging partial liver grafts, and (iii) each graft is anatomically appropriate for APOLT. In addition, we evaluate it with a focus on selection of cases, designing of graft sizes, and surgical techniques for reciprocal APOLT.
- Published
- 2016