an inborn error of metabolism in which the lack of certain enzymes leads to an inability to metabolize the amino acid lysine; characterized by muscular weakness and mental retardation
English Journal
[Hyperlysinemia and hyperammonemia].
Rabier D1, Parvy P, Bardet J, Kamoun P.
Annales de biologie clinique.Ann Biol Clin (Paris).1991;49(1):45-8.
A quite important increase of plasma lysine was often reported in different cases of hyperammonemia. This retrospective study of patients with different types of hyperammonemia shows that hyperlysinemia is not automatically associated to hyperammonemia (lysinemia is expressed as the percent of total
Intravenous loading with arginine-hydrochloride and ornithine-aspartate in siblings of two families, presenting a familial neurological syndrome associated with cystinuria.
Strauven T, Mardens Y, Clara R, Terheggen H.
Biomedicine / [publiée pour l'A.A.I.C.I.G.].Biomedicine.1976 Jun;24(3):191-9.
1. Arginine-hydrochloride and ornithine-aspartate solutions have been infused intravenously to children of two families. Three children of the WOL. family are affected with hyperargininemia and hyperammonemia, due to a lack of arginase. They present a secondary cystine-lysinuria. The three WIL. sibl
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