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肾衰竭背景下的迟发性皮肤卟啉病。对肾移植的反应。

Porphyria cutanea tarda in the setting of renal failure. Response to renal transplantation.

作者信息

Stevens B R, Fleischer A B, Piering F, Crosby D L

机构信息

Department of Dermatology, Medical College of Wisconsin, Milwaukee 53226.

出版信息

Arch Dermatol. 1993 Mar;129(3):337-9.

PMID:8095384
Abstract

BACKGROUND

Porphyria cutanea tarda (PCT) and similar vesiculobullous disorders have been described with increased frequency in patients with chronic renal failure undergoing long-term hemodialysis. Descriptions of over 20 cases of hemodialysis-related PCT have appeared in the literature. The precise cause of PCT in chronic renal failure is unknown, and the treatment of PCT occurring in this setting is complex and often ineffective.

OBSERVATIONS

We describe a patient who developed hemodialysis-related PCT, which, in this patient, was refractory to various treatment modalities. This patient eventually went on to receive a renal transplant and all symptoms of PCT resolved with normalization of urinary porphyrin levels.

CONCLUSIONS

We propose that in PCT refractory to the currently available treatment modalities occurring after the development of chronic renal failure, renal transplantation is indicated as a potentially efficacious therapy.

摘要

背景

迟发性皮肤卟啉病(PCT)及类似的水疱大疱性疾病在接受长期血液透析的慢性肾衰竭患者中出现的频率有所增加。文献中已出现20多例与血液透析相关的PCT的描述。慢性肾衰竭中PCT的确切病因尚不清楚,在此情况下发生的PCT的治疗复杂且往往无效。

观察结果

我们描述了一名发生与血液透析相关的PCT的患者,该患者对各种治疗方式均无效。该患者最终接受了肾移植,PCT的所有症状随着尿卟啉水平恢复正常而消失。

结论

我们提出,对于慢性肾衰竭发生后对当前可用治疗方式无效的PCT,肾移植可作为一种潜在有效的治疗方法。

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