Doss M, Verspohl F
Klin Wochenschr. 1981 Jul 1;59(13):727-35. doi: 10.1007/BF01721260.
The "glucose effect" was investigated in human acute hepatic porphyrias (acute intermittent porphyria, variegate porphyria, coproporphyria and porphobilinogen synthase defect porphyria) and in avian liver cells. 8 patients (7 women) with acute abdominal-neurological porphyria syndrome and 3 patients (2 women) in the remission phase were treated with high carbohydrate intake (approximately 500 g/24 h), mainly in form of intravenous glucose infusions. The biochemical response with a decrease of metabolites of porphyrin biosynthesis was highly significant, accompanied by clinical improvement in 10 courses of 9 patients. Two patients with delayed detection of the disease under the condition of Landry paralysis died after temporary clinical improvement due to ventricular arrythmias in one case and septicemia in the other. The importance of early diagnosis and therapy, and omission of drugs and alcohol cannot be overemphasized. Complementary studies show the "glucose effect" in drug -mediated induction of porphyrin synthesis in liver cells grown in culture: delta-Aminolevulinic acid synthase and protoporphyrin synthesis are repressed.
在人类急性肝卟啉病（急性间歇性卟啉病、混合型卟啉病、粪卟啉病和胆色素原合酶缺陷型卟啉病）以及禽肝细胞中研究了“葡萄糖效应”。8例（7名女性）患有急性腹部 - 神经卟啉病综合征的患者和3例（2名女性）处于缓解期的患者接受了高碳水化合物摄入（约500克/24小时）治疗，主要形式为静脉输注葡萄糖。卟啉生物合成代谢产物减少的生化反应非常显著，9例患者的10个疗程均伴有临床改善。2例在Landry麻痹情况下疾病检测延迟的患者，1例因室性心律失常，另1例因败血症，在暂时临床改善后死亡。早期诊断和治疗以及避免使用药物和酒精的重要性再怎么强调也不为过。补充研究表明，在培养的肝细胞中，药物介导的卟啉合成诱导存在“葡萄糖效应”：δ-氨基乙酰丙酸合酶和原卟啉合成受到抑制。
