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迟发性皮肤卟啉症治疗进展。皮下缓慢输注去铁胺的有效性。

Advances in the treatment of porphyria cutanea tarda. Effectiveness of slow subcutaneous desferrioxamine infusion.

作者信息

Gibertini P, Rocchi E, Cassanelli M, Pietrangelo A, Ventura E

出版信息

Liver. 1984 Aug;4(4):280-4. doi: 10.1111/j.1600-0676.1984.tb00938.x.

Abstract

The authors present the results of long-term subcutaneous desferrioxamine (DFX) infusion in 16 porphyria cutanea tarda (PCT) patients who cannot undergo repeated phlebotomies because of severe liver damage, haemolytic anemia, cardiovascular impairment or pulmonary and bone tuberculosis. They employed an automatic, portable syringe pump for subcutaneous infusion (8-10 h) to overcome the short half-life of the drug. Photodynamic cutaneous lesions and hyperpigmentation quickly disappeared (2-3 months). Uroporphyrin excretion sharply decreased and normalized within 3-12 months. Also, serum iron and ferritin, as well as liver function, showed a significant improvement. The authors therefore propose subcutaneous DFX therapy in PCT treatment when phlebotomy is contraindicated.

摘要

作者介绍了16例迟发性皮肤卟啉病(PCT)患者长期皮下注射去铁胺(DFX)的结果。这些患者因严重肝损伤、溶血性贫血、心血管功能损害或肺及骨结核而无法接受反复放血治疗。他们采用自动便携式注射器泵进行皮下输注(8 - 10小时),以克服药物半衰期短的问题。光动力皮肤病变和色素沉着迅速消失(2 - 3个月)。尿卟啉排泄在3 - 12个月内急剧下降并恢复正常。此外,血清铁、铁蛋白以及肝功能均有显著改善。因此,作者建议在放血治疗禁忌时,采用皮下注射DFX疗法治疗PCT。

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