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急性间歇性卟啉病的活性炭血液灌流和血液透析

Charcoal haemoperfusion and haemodialysis in acute intermittent porphyria.

作者信息

Laiwah A C, Junor B, MacPhee G J, Thompson G G, McColl K E

出版信息

Br Med J (Clin Res Ed). 1983 Dec 10;287(6407):1746-7. doi: 10.1136/bmj.287.6407.1746.

Abstract

Charcoal haemoperfusion has been advocated as a means of removing delta aminolaevulinic acid, which accumulates in attacks of acute intermittent porphyria. A woman presented with acute intermittent porphyria unresponsive to conventional treatment and with pain that was difficult to control. Charcoal haemoperfusion was performed in series with haemodialysis for two hours daily on four consecutive days. Although during this treatment serum and urinary concentrations of delta aminolaevulinic acid and porphobilinogen were considerably reduced, they had returned to pretreatment values 24 hours after the end of treatment. Abdominal pain was not relieved. Although a longer course of treatment might have had a more favourable outcome, this seems unlikely in view of the rapid rebound of serum concentration of delta amino-laevulinic acid after each haemoperfusion.

摘要

活性炭血液灌流已被提倡作为清除δ-氨基乙酰丙酸的一种方法,δ-氨基乙酰丙酸在急性间歇性卟啉病发作时会蓄积。一名患有急性间歇性卟啉病的女性患者,对传统治疗无反应且疼痛难以控制。连续四天每天进行两小时的活性炭血液灌流与血液透析串联治疗。尽管在治疗期间,血清和尿液中δ-氨基乙酰丙酸和卟胆原的浓度大幅降低,但在治疗结束24小时后又恢复到治疗前水平。腹痛并未缓解。尽管更长疗程的治疗可能会有更有利的结果,但鉴于每次血液灌流后δ-氨基乙酰丙酸血清浓度迅速反弹,这种可能性似乎不大。

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Haemoperfusion in acute intermittent porphyria.急性间歇性卟啉病中的血液灌流
Br Med J (Clin Res Ed). 1984 May 12;288(6428):1458. doi: 10.1136/bmj.288.6428.1458.

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