フェニールケトン尿症に対するインスリンの効果 第二編 血中遊離アミノ酸に対する影響

1. 対照正常人6例とPKU患者6例について,インスリン投与前と投与120分後の血中遊離アミノ酸の変化を検討した. 2. 対照正常人では, Aspが2例で増加したほかはすべてのアミノ酸が減少し, Met, Leu, Arg, Ileの順に減少率が大であった. 3. PKU患者では, Pheは4例, Tyrは全例で減少した. 4. PKU患者でGlu, Asp, Arg, His, Tau, Trp, Orn, Proの増加例があった.

Free amino acids in sera after insulin injection were studied using six PKU subjects and six normal subjects. Blood samples were collected prior to the insulin injection and 120 minutes after insulin injection. The results were as follows; 1. Serum free amino acid level of normal subjects except aspartic acid level were all decreased after insulin injection. 2. Serum levels of free tyrosine, leucine, isoleucine, valine, alanine, glutamic acid, threonine, arginine, lysine and proline of PKU subjects were significantly lower than normal level. 3. Phenylalanine level is decreased in four PKU cases 120 minutes after insulin injection. Tyrosine level decreased in all PKU cases 120 minutes after insulin injection. 4. The levels of glutamic acid (4 cases), aspartic acid (3 cases), proline (2 cases), tryptophan (1 case), taurine (1 case), arginine (1 case), histidine (1 case) and ornithine (1 case) of PKU subjects were increased after insulin injection.