Soliman S I, Hafez M, Ibrahim A, Shaaban N A, Hafez T A, Mehareb S W
Gaz Egypt Paediatr Assoc. 1976 Jul-Oct;24(3-4):217-35.
7 infants diseased with Acrodermatitis enteropathica and 10 normal controls were included in this study. The values of anthranilic acid glucuronide, 6- aminohippuric, anthranilic acid, N-acetyl Kneurine, Kneurine and 30 H Kneurenine, were estimated in mg/24 hours urine, both basal and after tryptophane load. In addition, histopathological and histochemical studies for lactase, succinic dehydrogenase, alkaline phosphatase, acid phosphatase, and alpha-non-specific esterases activities were done for the intestinal mucosal biopsies. All the previous investigations were then repeated after two months treatment with 500 mg/day diiodohydroxyquinoline. The tryptophan metabolites were significantly low in the diseased infants, both basal and after tryptophan load. Moreover, the intestinal enzymes activities were altered. After 2 months treatment with diiodohydroxyquinoline the diseased infants became clinically improved, tryphtophan metabolites became normal, but the activities of the intestinal enzymes were not altered. The biochemical and histochemical findings were discussed, giving the possibility of competitive inhibition of the diiodohydroxyquinolines and the by-product 8 OH Quinololic acid resulting in more degradation of Kneurine and 3 OH Kneurenine to nicotinamide adenine dinucleotide.
本研究纳入了7例患肠病性肢端皮炎的婴儿和10名正常对照。测定了基础状态下以及色氨酸负荷后24小时尿液中邻氨基苯甲酸葡萄糖醛酸、6-氨基马尿酸、邻氨基苯甲酸、N-乙酰神经氨酸、神经氨酸和30H神经氨酸的含量，单位为mg/24小时尿液。此外，对肠黏膜活检组织进行了乳糖酶、琥珀酸脱氢酶、碱性磷酸酶、酸性磷酸酶和α-非特异性酯酶活性的组织病理学和组织化学研究。在用500mg/天的双碘喹啉治疗两个月后，重复了所有先前的研究。患病婴儿基础状态下以及色氨酸负荷后的色氨酸代谢产物均显著降低。此外，肠道酶活性发生了改变。用双碘喹啉治疗2个月后，患病婴儿临床症状改善，色氨酸代谢产物恢复正常，但肠道酶活性未改变。对生化和组织化学结果进行了讨论，提出了双碘喹啉及其副产物8-羟基喹啉酸可能存在竞争性抑制作用，导致神经氨酸和3-羟基神经氨酸更多地降解为烟酰胺腺嘌呤二核苷酸的可能性。
