Non-hepatic hyperammonaemia: an important, potentially reversible cause of encephalopathy

The clinical syndrome of encephalopathy is most often encountered in the context of decompensated liver disease and the diagnosis is usually clear cut. Non-hepatic causes of encephalopathy are rarer and tend to present to a wide range of medical specialties with variable and episodic symptoms. Delay...

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Bibliographic Details
Published in:Postgraduate medical journal 2001-11, Vol.77 (913), p.717-722
Main Authors: Hawkes, N D, Thomas, G A O, Jurewicz, A, Williams, O M, Hillier, C E M, McQueen, I N F, Shortland, G
Format: Article
Language:English
Subjects:
Abdomen
Adult
Ammonia
Biological and medical sciences
Biopsy
Brain Diseases, Metabolic - etiology
Case Reports
Causes of
Child
Constipation
Constipation - chemically induced
Constipation - complications
Constipation - surgery
Electrolytes
Encephalopathy
Gastrointestinal Hemorrhage - complications
Gastrointestinal Hemorrhage - surgery
Glucose
Health aspects
Hospitals
Humans
hyperammonaemia
Hyperammonemia - complications
Liver cirrhosis
Liver diseases
Male
Medical sciences
Middle Aged
Nervous system involvement in other diseases. Miscellaneous
Neurology
Nitrogen
NMR
Nuclear magnetic resonance
Ornithine Carbamoyltransferase Deficiency Disease - complications
Ornithine Carbamoyltransferase Deficiency Disease - diet therapy
Pedigree
Portasystemic Shunt, Surgical
portosystemic venous shunt
Proteins
Rodents
Sodium
Thrombosis
Tomography
Treatment Outcome
urea cycle defect
Veins & arteries
Ventilation
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Summary:The clinical syndrome of encephalopathy is most often encountered in the context of decompensated liver disease and the diagnosis is usually clear cut. Non-hepatic causes of encephalopathy are rarer and tend to present to a wide range of medical specialties with variable and episodic symptoms. Delay can result in the development of potentially life threatening complications, such as seizures and coma. Early recognition is vital. A history of similar episodes or clinical risk factors and early assessment of blood ammonia levels help establish the diagnosis. In addition to adequate supportive care, investigation of the underlying cause of the hyperammonaemia is essential and its reversal, where possible, will often result in complete recovery. Detection of an unborn error of metabolism should lead to the initiation of appropriate maintenance therapy and genetic counselling.
ISSN:0032-5473
1469-0756
DOI:10.1136/pmj.77.913.717