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去铁胺成功治疗血液透析相关迟发性皮肤卟啉病

Successful treatment of hemodialysis-related porphyria cutanea tarda with deferoxamine.

作者信息

Stockenhuber F, Kurz R, Grimm G, Moser G, Balcke P

机构信息

Department of Renal Disease and Hemodialysis, Vienna University School of Medicine, Austria.

出版信息

Nephron. 1990;55(3):321-4. doi: 10.1159/000185983.

Abstract

End-stage renal failure and long-term hemodialysis treatment promote the development of genetically conditioned porphyria cutanea tarda (PCT). The clinical manifestation is triggered off by unknown factors coexisting with renal insufficiency and hemodialysis. Iron overload is often associated with the disease and is thought to play a key role in its pathogenesis. Iron removal by deferoxamine infusions is regarded as the treatment of choice for patients who cannot undergo repeated phlebotomy procedures and has been successfully used in patients with normal renal function. We report a case of hemodialysis-related PCT and iron overload in whom repeated venesections were contraindicated on account of severe anemia and treatment with deferoxamine led to a striking improvement of symptoms.

摘要

终末期肾衰竭和长期血液透析治疗会促使遗传性迟发性皮肤卟啉症(PCT)的发展。其临床表现由与肾功能不全和血液透析并存的未知因素引发。铁过载常与该疾病相关,且被认为在其发病机制中起关键作用。对于无法进行反复放血操作的患者,静脉输注去铁胺去除铁被视为首选治疗方法,且已成功应用于肾功能正常的患者。我们报告一例与血液透析相关的PCT和铁过载病例,该患者因严重贫血而禁忌反复放血,而去铁胺治疗使症状显著改善。

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