Leukocyte Urea Cycle Enzymes in Hyperammonemia
نویسندگان
چکیده
منابع مشابه
Hyperammonemia from a Urea Cycle Disorder Presenting in Adulthood
OBJECTIVE: The aim of this report is to describe a patient with late presentation of carbamyl phosphate synthetase I (CPS-I, EC 6.3.4.16) deficiency, a rare urea cycle deficiency, and to facilitate recognition and treatment of patients presenting with encephalopathy and hyperammonemia in a critical care setting. DESIGN: Case Report. SETTING: Intensive care unit of Saint Mary’s Hospital, Mayo Cl...
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The presence of all five enzymes of the ornithine-urea cycle has been demonstrated in the liver of the African lungfish Protopterus aethiopicus. Levels of activity of the rate-limiting enzymes, carbamoyl phosphate synthetase and argininosuccinate synthetase, are similar to those in the premetamorphic tadpole of Rana catesbeiana and considerably lower than the levels reported for other ureotelic...
متن کاملAdaptive characteristics of urea cycle enzymes in the rat.
The response of the mammal to varying dietary protein intake under conditions in which amino acid and energy requirements are adequately satisfied is a form of adaptation involving the utilization of varying proportions of carbohydrate and amino acids as sources of energy, and is therefore analogous to the adaptation seen in bacteria on change in form of energy source (1). The amino nitrogen re...
متن کاملHyperammonemia due to urea cycle disorders: a potentially fatal condition in the intensive care setting
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 ur...
متن کاملA Case of Suspected Urea Cycle Dysfunction in a Patient with Unexplained Hyperammonemia
Urea cycle disorders are inherited deficiencies of the enzymes involved in the cellular excretion of excess ammonia produced during protein metabolism. Hyperammonemia associated with these disorders is usually manifested by decreased level of consciousness, irritability, seizures, vomiting, and poor feeding. Although the majority of recognized patients are children, a delayed presentation is se...
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ژورنال
عنوان ژورنال: Pediatric Research
سال: 1975
ISSN: 0031-3998,1530-0447
DOI: 10.1203/00006450-197506000-00004