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Biomedical subjects

F Lavinha

Publications and source records attributed to F Lavinha.

7 recordsLinked to original sources
PubMed1976

Excretion of guanidino-derivates in urine of hyperargininemic patients.

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.

Adult↗
PubMed1975

Unsuccessful trial of gene replacement in arginase deficiency.

The Shope virus following cutaneous inoculation produces skin papillomas in rabbits and induces a virus-coded arginase in the squameous epithelium of these papillomas. The only discernible effect of the virus inoculated intravenously in rabbits and other animals was a decrease of the serum arginine concentration. Intravenous injection in 3 hyperargininemic patients, however, did not influence the underlying metabolic disease.

Amino Acid Metabolism, Inborn Errors↗
PubMed1975

Familial hyperargininaemia.

A third case of hyperargininaemia occurring in one family was studied from birth. In cord blood serum arginine concentration was only slightly raised, but arginase activity in red blood cell haemolysates was very low. In the urine on day 2 a typical cystinuria pattern was present. Arginine concentration in serum increased to 158 mumol/100 ml on the 41st day of life. Later determinations of the arginase activity in peripheral blood showed values below the sensitivity of the method. Blood ammonia was consistently high, and cystinuria was present. The enzymatic defect was further displayed by intravenous loading tests with arginine. Serum urea values were predominantly normal or near the lower limit of normal, suggesting the presence of other metabolic pathways of urea synthesis. In urine there was no excretion of guanidinosuccinic acid, while the excretion of other monosubstituted guanidine derivatives was increased, pointing to a connexion with hyperargininaemia. Owing to parental attitude, a low protein diet (1-5 g/kg) was introduced only late. The infant developed severe mental retardation, athetosis, and spasticity.

Amino Acids↗