New progress in liver transplantation for ornithine transcarbamylase deficiency

doi:10.3877/cma.j.issn.1674-3903.2023.03.010

Abstract

Abstract:

Ornithine transcarbamylase (OTC) deficiency (OTCD) is an X-linked genetic disorder caused by a genetic defect of OTC that results in disorders of ammonia metabolism, leading to recurrent hyperammonemia and neurological damage. For patients failed with conservative treatment, liver transplantation is currently the only curative method to correct the patient′s metabolic crisis and improve long-term survival quality. This article reviews the research progress of liver transplantation for OTCD in three aspects: indications and timing of liver transplantation, surgical approach and donor selection, and long-term postoperative outcomes.

Key words: Ornithine transcarbamylase deficiency, Liver transplantation, Neurodevelopmental injury

Dongni Lin, Ping Wan, Qiang Xia. New progress in liver transplantation for ornithine transcarbamylase deficiency[J]. Chinese Journal of Transplantation(Electronic Edition), 2023, 17(03): 180-185.

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