实用器官移植电子杂志 ›› 2023, Vol. 11 ›› Issue (4): 301-304.DOI: 10.3969/j.issn.2095-5332.2023.04.004

• 论著 • 上一篇    下一篇

肝移植治疗鸟氨酸氨甲酰基转移酶缺乏症 5 例 临床诊疗分析

刘单,孙延东,王峰,张慧,吴斌,滕大洪,蔡金贞
  

  1. 青岛大学附属医院儿童器官移植科,山东 青岛 266000

  • 出版日期:2023-07-20 发布日期:2023-09-12

Clinical diagnosis and treatment analysis of 5 cases of ornithine aminotransferase deficiency treated with liver transplantation 

Liu Dan,Sun Yandong,Wang Feng,Zhang Hui,Wu Bin,Teng Dahong,Cai Jinzhen.    

  1. Department of Child OrganTransplantation,the Affiliated Hospital of Qingdao University,Qingdao 266000,Shandong,China.

  • Online:2023-07-20 Published:2023-09-12

摘要:

目的 评估肝移植治疗鸟氨酸氨甲酰基转移酶缺乏症(ornithine transcarbamylase deficiency,OTCD)的临床效果。 方法 本研究回顾性分析本中心收治的 5 例 OTCD 所致高氨血症接受肝移植术患儿资料,分析手术相关并发症,术后血氨水平、肝功能恢复情况。 结果 5 例患儿均接受肝移植治疗,1 例因胆瘘行二次胆肠吻合术,1 例因原发性移植物无功行二次肝移植术。5 例患儿术后血氨均迅速下降,术后14 d 内均恢复至正常水平,肝功能 9 d 内恢复正常水平,远期随访神经系统损伤部分缓解。 结论 肝移植对于 OTCD 患者的治疗是安全有效的,达到完全治愈,并可改善患者的远期生活质量。

关键词: 鸟氨酸氨甲酰基转移酶缺乏症 , ;肝移植 ,  , 儿童

Abstract:

Objective To evaluate the clinical efficacy of liver transplantation in the treatment of ornithinetranscarbamylase deficiency(OTCD). Methods This study retrospectively analyzed 5 children with OTCD induced hyperammonemia who underwent liver transplantation in our center,surgical related complications,postoperative blood ammonia levels,and liver function recovery were analyzed. Results All 5 patients received liver transplantation treatment,with 1 patient underwent secondary cholangiojejunostomy due to biliary fistula and 1 patient underwent secondary liver transplantation due to primary graft failure. All 5 patients had a rapid decrease in postoperative blood ammonia levels,which returned to normal levels within 14 days after surgery. Liver functionreturned to normal levels within 9 days,and partial relief of neurological damage was observed during long-termfollow-up. Conclusion Liver transplantation is a safe and effective treatment for OTCD patients,complete cure and improved their long-term quality of life could be achieved. 

Key words:

Ornithine aminotransferase deficiency, Liver transplantation, Children