Marchiori P E, de Assis J L, Scaff M, Do Serro-Azul L G, Moraes E C, Bacheschi L A, Yassuda N
Rev Bras Pesqui Med Biol. 1979 Sep;12(4-5):325-33.
A young woman with acute intermittent porphyria in profound relapse and severe nervous and respiratory involvement was treated by intravenous infusions of hematin, followed by improvement of symptoms. The diet with high carbohydrate and protein content and a B-adrenergic blocking agent, not showed any beneficial effect on acute attack. The infusion of hematin was followed by a lowering effect on urine porphybilinogen and delta-aminolevulinic acid and clinical improvement. The return to normal of porphyrin precursors in the urine was accompanied by almost complete clinical remission. The relationship of remission and repression of delta-aminolevulinic acid, decrease of urinary levels of porphyrin precursors, pulmonaries tests and electromyograms, were discussed.
一名患有急性间歇性卟啉症且病情严重复发、伴有严重神经和呼吸系统受累的年轻女性，通过静脉输注血红素进行治疗，随后症状得到改善。高碳水化合物和蛋白质含量的饮食以及一种β-肾上腺素能阻滞剂对急性发作未显示出任何有益效果。输注血红素后，尿卟啉原和δ-氨基乙酰丙酸水平降低，临床症状改善。尿中卟啉前体恢复正常的同时，临床几乎完全缓解。文中讨论了缓解与δ-氨基乙酰丙酸抑制、尿卟啉前体水平降低、肺部检查和肌电图之间的关系。
