Transplantation urea cycle disorders

Whitington PF, Alonso EM, Boyle JT, Molleston JP, Rosenthal P, Emond JC, et al. Liver transplantation for the treatment of urea cycle disorders. J Inher MetabDis 1998 21 112-8. 

Perito ER, et al. Pediatric liver transplantation for urea cycle disorders and organic acidemias united network for organ sharing data for 2002-2012. Liver Tianspl. 2014 20(l) 89-99. 

Kim IK, et al. Liver transplantation for urea cycle disorders in pediatric patients a single-center experience. Pediatr Transplant. 2013 17(2) 158-67. 

The primary function of the urea cycle is to rid the body of waste nitrogen. Deficiency in the activity of any of the six enzymes in the urea cycle may result in the accumulation of ammonia, often to toxic concentrations. Treatment involves restricting protein, preventing catabolism, supplementing amino acids that are normally produced by the urea cycle, and promoting the excretion of nitrogen via alternative pathways. Outcomes are guarded and appear to be better for patients identified by NBS compared to patients identified clinically. Liver transplantation is a treatment option, especially for patients with a severe form of the disorder. 

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