Results 151 to 160 of about 23,446 (182)
Some of the next articles are maybe not open access.
1990
Patients with urea cycle disorders can present with symptoms from birth to adulthood, depending on the degree of enzyme defect and nitrogen load. Levels of ammonia in the blood should be determined in any newborn with a course which is at variance with the expectation of the clinician especially if symptoms such as loss of appetite, vomiting, seizures,
openaire +1 more source
Patients with urea cycle disorders can present with symptoms from birth to adulthood, depending on the degree of enzyme defect and nitrogen load. Levels of ammonia in the blood should be determined in any newborn with a course which is at variance with the expectation of the clinician especially if symptoms such as loss of appetite, vomiting, seizures,
openaire +1 more source
2010
Abstract The urea cycle is a series of steps required to generate urea from nitrogen produced by protein catabolism. The cycle was first described in 1932 by Krebs and Henseleit (Krebs and Henseleit 1932). Six enzymes and two transporters are necessary for urea cycle activity. Specific deficiencies have been described with each of these.
openaire +1 more source
Abstract The urea cycle is a series of steps required to generate urea from nitrogen produced by protein catabolism. The cycle was first described in 1932 by Krebs and Henseleit (Krebs and Henseleit 1932). Six enzymes and two transporters are necessary for urea cycle activity. Specific deficiencies have been described with each of these.
openaire +1 more source
Okonkwo I, Stuart G, Ekasumara NF, Huncke TK: Urea cycle disorders, 2021
Okonkwo, Ijeoma +3 more
openaire +2 more sources
Okonkwo, Ijeoma +3 more
openaire +2 more sources
Problems in the management of urea cycle disorders
Molecular Genetics and Metabolism, 2004Several recent reviews describe the management of urea cycle disorders. There is much agreement on diet, alternative pathway therapy, maintenance of arginine and ornithine levels in acute and chronic management, sick-day regimens, and some aspects of monitoring. However, differences remain in several areas, and physicians at most treatment centers have
openaire +2 more sources
Biochemical Aspects of Urea Cycle Disorders
Pediatrics, 1981In this paper I will review the biochemistry of the urea cycle of mammalian liver and point out the important clinical implications of this biochemistry. Second, I will consider some problems that arise in the use of human liver enzyme assays for evaluating deficiencies of urea cycle enzymes.
openaire +2 more sources
2000
Five inherited disorders of the urea cycle are now well described. These are characterised by hyperammonaemia and disordered amino-acid metabolism. The presentation is highly variable: those presenting in the newborn period usually have an overwhelming illness that rapidly progresses from poor feeding, vomiting, lethargy or irritability and tachypnoea ...
openaire +1 more source
Five inherited disorders of the urea cycle are now well described. These are characterised by hyperammonaemia and disordered amino-acid metabolism. The presentation is highly variable: those presenting in the newborn period usually have an overwhelming illness that rapidly progresses from poor feeding, vomiting, lethargy or irritability and tachypnoea ...
openaire +1 more source
Hereditary Metabolic Disorders of the Urea Cycle
1971Publisher Summary Urea is the main end product of nitrogen metabolism. It is formed from the ammonia arising from the metabolism of the amino acids of protein by a sequence of five reactions, four of which comprise the urea cycle proper. The end result is the conversion of ammonia into urea, with the reformation of the individual reactants of the ...
openaire +2 more sources
Urea cycle activation triggered by host-microbiota maladaptation driving colorectal tumorigenesis
Cell Metabolism, 2023Tianying Tong, Linhua Ji, Baoqin Xuan
exaly