De Antoni A, Muggeo M, Costa C, Allegri G, Crepaldi G
Acta Vitaminol Enzymol. 1976;30(4-6):134-9.
The tryptophan metabolism "via" kynurenine was studied in five patients with scleroderma after aminoacid loading. Four of these patients had abnormal tryptophan metabolism, characterized by a large urinary excretion of kynurenine and kynurenic acid in two cases, of kynurenine, 3-hydroxykynurenine and kynurenic acid in one case and of 3-hydroxyanthranilic acid in another case and generally a reduced excretion of xanthurenic acid and its 8-methyl ether in comparison with a group of healthy controls. Only two of the four patients had a normal response to tryptophan loading after pyridoxine administration, while no one of these responded to nicotinamide supplementation. But the simultaneous administration of pyridoxine and nicotinamide to three of these patients normalized the excretory picture after tryptophan loading. This suggested the presence of a combined vitamin deficiency in seleroderma. As four out of five patients showed total excretory values of kynurenine, kynurenic acid and acetylkynurenine higher than that of the controls, the sum of these values might be considered as a characteristic index of scleroderma.
对5例硬皮病患者在氨基酸负荷后通过犬尿氨酸途径的色氨酸代谢进行了研究。其中4例患者色氨酸代谢异常,表现为:2例患者尿中犬尿氨酸和犬尿酸大量排泄;1例患者尿中犬尿氨酸、3 - 羟基犬尿氨酸和犬尿酸排泄增加;另1例患者尿中3 - 羟基邻氨基苯甲酸排泄增加;与一组健康对照相比,尿中黄尿酸及其8 - 甲醚的排泄通常减少。4例患者中只有2例在给予吡哆醇后对色氨酸负荷有正常反应,而这些患者中无人对补充烟酰胺有反应。但对其中3例患者同时给予吡哆醇和烟酰胺后,色氨酸负荷后的排泄情况恢复正常。这提示硬皮病患者存在复合维生素缺乏。由于5例患者中有4例的犬尿氨酸、犬尿酸和乙酰犬尿氨酸的总排泄值高于对照组,这些值的总和可被视为硬皮病的一个特征性指标。