Molecular genetics and metabolism, 100 Suppl 1, S31-S36 (2010-02-24)
The paucity of hyperammonemic crises together with spasticity, only seen in human arginase I deficient patients and not in patients with other urea cycle disorders, forces a search for candidates other than ammonia to associate with the pathophysiology and symptomatology.
Serum levels of twelve guanidino compounds (GCs) and nerve conduction velocities were determined in a dialyzed renal insufficient pediatric population. Two dialytic groups were considered: one subjected to hemodialysis (HD, 11 patients) and one subjected to continuous cycle peritoneal dialysis
Clinica chimica acta; international journal of clinical chemistry, 157(2), 143-150 (1986-06-15)
The concentrations of guanidino compounds in blood are raised in uraemic patients and may have toxic effects. The concentrations of 13 guanidino compounds in serum were measured in 29 patients with chronic renal failure treated by chronic intermittent haemodialysis using
Binding of methylglyoxal to albumin and formation of fluorescent adducts. Inhibition by arginine, N-alpha-acetylarginine and aminoguanidine.
T Selwood et al.
Biochemical Society transactions, 21(2), 170S-170S (1993-05-01)
Hyperargininemia is a metabolic disorder biochemically characterized by tissue accumulation of arginine and other guanidino compounds. Convulsions, lethargy and psychomotor delay or cognitive deterioration are predominant clinical features of this disease. Although neurologic symptoms predominate in this disorder, their pathophysiology
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