title: Noncirrhotic hyperammonaemic encephalopathy.
abstract: 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.
authors: Department of Internal Medicine A and Rabin Medical Center, Beilinson Hospital, Petah Tiqwa, Israel.
authors: Ido
authors: I
authors: Laish
authors: Ziv
authors: Z
authors: Ben Ari
mesh_terms: D000328:Adult; D000641:Ammonia / Q000097:blood; D015415:Biomarkers / Q000097:blood; D001928:Brain Diseases, Metabolic / Q000097:blood / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology / Q000628:therapy; D042241:Early Diagnosis; D006801:Humans; D022124:Hyperammonemia / Q000097:blood / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology / Q000628:therapy; D008111:Liver Function Tests; D020258:Neurotoxicity Syndromes / Q000097:blood / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology / Q000628:therapy; D011237:Predictive Value of Tests; D011379:Prognosis; D018570:Risk Assessment; D012307:Risk Factors
chemical_list: D015415:Biomarkers; D000641:Ammonia