title: [Liver transplantation for the treatment of hyperammonemia due to urea cycle disorder: report of four cases].
abstract: OBJECTIVE
  To analyze clinical efficacy and prognosis of liver transplantation in children with hyperammonemia caused by urea cycle disorders.
  
  
  METHODS
  A retrospective analysis was performed on the occurrence of disease, operation and the follow-up post liver transplantation in 4 patients with urea cycle disorders who underwent liver transplantation during June 2001 to May 2014.
  
  
  RESULTS
  Four girls were diagnosed with ornithine carbamoyl transferase deficiency by genetic test. They had the clinical onset at the age of 1.5 to 3.0 years. Liver transplantation had been performed at their age of 53.9 months, 40.6 months, 40.3 months and 22.8 months, respectively. The grafts of case 1 and case 2 were from left lateral lobe of liver of cadaveric donor, the graft of case 3 was from left lateral lobe of liver of a living donor, the graft of case 4 was a whole liver of a dead child. The liver function of 4 patients gradually returned to normal, blood ammonia levels were normal and restored the normal diet, 4 children were discharged on postoperative 25-30 days. Regular follow-up was done, the liver function, biochemical features and growth status have been followed up for 162.2 months, 124.2 months, 12.0 months and 4.8 months after liver transplantation, respectively. Now, all the four cases are healthy and growth is normal.
  
  
  CONCLUSIONS
  Liver transplantation is an important way to the patients with severe hyperammonemia caused by urea cycle disorders. In this study, the patients with ornithine carbamoyl transferase defect got satisfactory long-term outcome after liver transplantation.
authors: Department of General Surgery, Beijing Friendship Hospital, Capital Medical University, Beijing 100050, China. Email: zhu-zhijun@medmail.com.cn.
authors: Zhijun
authors: Z
authors: Zhu
authors: Liying
authors: L
authors: Sun
authors: Lin
authors: L
authors: Wei
authors: Wei
authors: W
authors: Qu
authors: Zhigui
authors: Z
authors: Zeng
authors: Ying
authors: Y
authors: Liu
authors: Liang
authors: L
authors: Zhang
authors: Enhui
authors: E
authors: He
authors: Dong
authors: D
authors: Wang
mesh_terms: D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D022124:Hyperammonemia / Q000209:etiology* / Q000601:surgery*; D008099:Liver; D008111:Liver Function Tests; D016031:Liver Transplantation; D019520:Living Donors; D020163:Ornithine Carbamoyltransferase Deficiency Disease / Q000150:complications*; D011379:Prognosis; D012189:Retrospective Studies; D014019:Tissue Donors