通过静脉输注血红素抑制急性间歇性卟啉病中卟啉前体的过度产生。
Repression of the overproduction of porphyrin precursors in acute intermittent porphyria by intravenous infusions of hematin.
作者信息
Bonkowsky H L, Tschudy D P, Collins A, Doherty J, Bossenmaier I, Cardinal R, Watson C J
出版信息
Proc Natl Acad Sci U S A. 1971 Nov;68(11):2725-9. doi: 10.1073/pnas.68.11.2725.
Abstract
In a patient with a severe attack of acute intermittent porphyria, hematin given intravenously caused marked diminution of serum delta-aminolevulinic acid and porphobilinogen. The decline of aminolevulinate was more rapid than that of porphobilinoge. After 2 days of hematin administration, about 5 days were required for delta-aminolevulinic acid, and 11 days for porphobilinogen to return to the concentrations that were detected before treatment. Urinary excretion of both compounds also decreased after hematin administration. Considerable amounts of porphobilinogen were also found in the cerebrospinal fluid of the patient.
摘要
在一名急性间歇性卟啉症严重发作的患者中，静脉注射血红素可使血清δ-氨基-γ-酮戊酸和卟胆原显著减少。δ-氨基-γ-酮戊酸的下降速度比卟胆原更快。给予血红素2天后，δ-氨基-γ-酮戊酸大约需要5天、卟胆原需要11天才能恢复到治疗前检测到的浓度。给予血红素后，这两种化合物的尿排泄量也减少。在该患者的脑脊液中也发现了相当数量的卟胆原。
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