高精氨酸血症患者治疗期间血浆、尿液和脑脊液中的胍基化合物。

Guanidino compounds in plasma, urine and cerebrospinal fluid of hyperargininemic patients during therapy.

作者信息

Marescau B, Qureshi I A, De Deyn P, Letarte J, Ryba R, Lowenthal A

出版信息

Clin Chim Acta. 1985 Feb 28;146(1):21-7. doi: 10.1016/0009-8981(85)90120-2.

Abstract

The concentrations of guanidino compounds were determined in urine, plasma and cerebrospinal fluid of two patients with hyperargininemia during dietary therapy. alpha-Keto-delta-guanidinovaleric acid, N-alpha-acetylarginine, argininic acid and gamma-guanidinobutyric acid were increased in urine. In plasma, these compounds together with creatine, guanidinoacetic acid, arginine and homoarginine were also increased. In cerebrospinal fluid, only arginine, homoarginine and argininic acid were increased. Trace amounts of alpha-keto-delta-guanidinovaleric acid were found in cerebrospinal fluid of the patient treated with only a low-arginine diet. The concentrations of guanidinosuccinic acid are decreased in urine, plasma and cerebrospinal fluid. During a low-arginine diet, together with sodium benzoate therapy, the plasma and cerebrospinal fluid arginine values returned to normal. There was also a normalization of plasma guanidinoacetic acid and a marked decrease in plasma N-alpha-acetylarginine and argininic acid.

摘要

在饮食治疗期间，测定了两名高精氨酸血症患者尿液、血浆和脑脊液中胍基化合物的浓度。尿液中α-酮-δ-胍基戊酸、N-α-乙酰精氨酸、精氨酸酸和γ-胍基丁酸增加。血浆中，这些化合物以及肌酸、胍基乙酸、精氨酸和高精氨酸也增加。脑脊液中，仅精氨酸、高精氨酸和精氨酸酸增加。在仅接受低精氨酸饮食治疗的患者脑脊液中发现了痕量的α-酮-δ-胍基戊酸。尿液、血浆和脑脊液中胍基琥珀酸的浓度降低。在低精氨酸饮食期间，联合苯甲酸钠治疗，血浆和脑脊液中的精氨酸值恢复正常。血浆胍基乙酸也恢复正常，血浆N-α-乙酰精氨酸和精氨酸酸显著降低。