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Papers of the Week

2022 Jun 26

World J Clin Cases



Liver transplantation for late-onset ornithine transcarbamylase deficiency: A case report.


Fu X-H, Hu Y-H, Liao J-X, Chen L, Hu Z-Q, Wen J-L, Chen S-L
World J Clin Cases. 2022 Jun 26; 10(18):6156-6162.
PMID: 35949846.


Ornithine transcarbamylase deficiency (OTCD) is an X-linked inherited disorder and characterized by marked elevation of blood ammonia. The goal of treatment is to minimize the neurological damage caused by hyperammonemia. OTCD can be cured by liver transplantation (LT). Post-transplant patients can discontinue anti- hyperammonemia agents and consume a regular diet without the risk of developing hyperammonemia. The neurological damage caused by hyperammonemia is almost irreversible.