Department of Internal Medicine A and Rabin Medical Center, Beilinson Hospital, Petah Tiqwa, Israel.
Liver Int. 2011 Oct;31(9):1259-70. doi: 10.1111/j.1478-3231.2011.02550.x. Epub 2011 Jun 21.
Adult hyperammonaemia is associated with severe liver disease in 90% of cases. In the remainder, noncirrhotic causes should be considered. Measurements of serum ammonia level must be part of the basic work-up in all patients presenting with encephalopathy of unknown origin, even when liver function is normal. Clinician awareness of noncirrhotic hyperammonaemic encephalopathy can contribute to early diagnosis and the initiation of sometimes life-saving treatment. This review focuses on the physiology, aetiology and underlying mechanisms of noncirrhotic hyperammonaemic encephalopathy and discusses the available treatment modalities.
成人高血氨症 90%与严重肝脏疾病相关。在其余病例中，应考虑非肝硬化病因。所有出现不明原因脑病的患者，即使肝功能正常，也应进行血清氨水平测量，这是基本检查的一部分。临床医生对非肝硬化性高血氨性脑病的认识有助于早期诊断和开始有时可救命的治疗。这篇综述重点讨论了非肝硬化性高血氨性脑病的生理学、病因和潜在机制，并讨论了现有的治疗方法。
