Wiechert P, Mortelmans J, Lavinha F, Clara R, Terheggen H G, Lowenthal A
J Genet Hum. 1976 Mar;24(1):61-72.
Quantitative determinations were made for seven guanidino-derivates in urine of patients affected with hyperargininemia, lysine cystinuria, healthy children and adults, goats and monkey. The amount of all seven guanidino-derivates excreted in the urine with the exception of guanidino-succinic acid was large in the case of hyperargininemic patients. Guanidino-succinic acid is found in traces in the urine of these patients, even after arginine loading tests. There is apparently no similarity concerning the guanidino-derivates between the patients with hyperargininemia, a pathological status, and the arginase deficiency in goats and monkeys, a physiological phenomenon.