Department of Surgery, Brigham and Women's Hospital, Boston, Massachusetts.
Department of Medicine, Renal Division, Brigham and Women's Hospital, Boston, Massachusetts.
Am J Transplant. 2019 Nov;19(11):3197-3201. doi: 10.1111/ajt.15545. Epub 2019 Sep 12.
A 72-year-old woman with end-stage kidney disease due to recurrent urinary tract infections and obstructive uropathy of a solitary kidney presented to our hospital for renal transplantation. She underwent successful transplantation of a deceased donor allograft, but developed acute mental status deterioration on the fifth postoperative day. Her serum ammonia was found to be markedly elevated to 447 μmol/L in the setting of normal hepatic function. She was treated with emergent dialysis and empiric antibiotics targeting urea-splitting organisms, and ultimately made a full neurologic recovery with stable renal allograft function. Noncirrhotic hyperammonemia (NCH) is an exceedingly rare clinical entity but seems to have a predilection for patients who have undergone solid organ transplantation. This report emphasizes the importance of rapid diagnosis and initiation of treatment for NCH, which is associated with a high rate of mortality and irreversible neurological morbidity. We outline the successful workup and management approach for this patient.
一位 72 岁老年女性，因反复尿路感染和孤立肾梗阻性尿路病导致终末期肾病，来我院行肾移植。她成功进行了尸体供者同种异体移植，但在术后第 5 天出现急性精神状态恶化。她的血清氨在肝功能正常的情况下显著升高至 447μmol/L。她接受了紧急透析和针对尿素分解菌的经验性抗生素治疗，最终肾功能稳定，神经功能完全恢复。非肝硬化性高血氨症（NCH）是一种极其罕见的临床实体，但似乎偏爱接受实体器官移植的患者。本报告强调了快速诊断和治疗 NCH 的重要性，因为它与高死亡率和不可逆转的神经发病率相关。我们概述了这位患者的成功检查和治疗方法。
