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2014 ; 2
(1
): 22
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Hyperammonemia due to urea cycle disorders: a potentially fatal condition in the
intensive care setting
#MMPMID25908985
Machado MC
; Pinheiro da Silva F
J Intensive Care
2014[]; 2
(1
): 22
PMID25908985
show ga
Disorders of the urea cycle are secondary to a defect in the system that converts
ammonia into urea, resulting in accumulation of ammonia and other products. This
results in encephalopathy, coma, and death if not recognized and treated rapidly.
Late-onset urea cycle disorders may be precipitated by acute disease and can be
difficult to recognize because patients are already ill. Diagnosis of urea cycle
disorders is based on clinical suspicion and determination of blood ammonia in
suspected patients with neurological symptoms in the intensive care setting.
Treatment is based on the removal of ammonia by dialysis or hemofiltration,
reduction of the catabolic state, abolishment of nitrogen administration, and use
of pharmacological nitrogen scavenging agents.