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迟发性皮肤卟啉症的肝脏铁过载与去铁胺治疗

Liver iron overload and desferrioxamine treatment of porphyria cutanea tarda.

作者信息

Rocchi E, Cassanelli M, Borghi A, Paolillo F, Pradelli M, Pellizzardi S, Vezzosi A, Gallo E, Baccarani Contri M, Ventura E

机构信息

Chair of Terapia Medica Sistematica, University of Modena, Italy.

出版信息

Dermatologica. 1991;182(1):27-31. doi: 10.1159/000247732.

Abstract

The aim of this paper is to evaluate invasive and non-invasive indices of iron store and compare the effectiveness of different ferrodepletive protocols in 150 patients with porphyria cutanea tarda (PCT). Iron removal was performed either by intensive phlebotomy (22 cases) or slow subcutaneous and high intravenous doses of desferrioxamine (18 and 5 cases, respectively), and several laboratory parameters were studied; among these, oligo-elements and urinary porphyrins (detected by HPLC) were taken into account before and after the treatments. Serum iron, transferrin saturation, ferritin (RIA) and nuclear magnetic resonance results were compared with invasive findings in order to detect the metal deposition in liver tissue (atomic absorption concentration, optic or electron-microscopic detection). Liver iron overload was observed in 95% of cases. Full normalization of the disease took place by all the treatments, even if it required slightly more time in the phlebotomy group. We may conclude that ferrodepletive treatments are highly effective in PCT and, considering the fact that siderosis and liver damage always accompany the disease, these treatments are proposed as first choice in such cases.

摘要

本文旨在评估150例迟发性皮肤卟啉病(PCT)患者铁储存的侵入性和非侵入性指标,并比较不同去铁方案的有效性。通过强化放血(22例)或皮下缓慢及静脉高剂量去铁胺(分别为18例和5例)进行铁清除,并研究了多个实验室参数;其中,在治疗前后考虑了微量元素和尿卟啉(通过高效液相色谱法检测)。将血清铁、转铁蛋白饱和度、铁蛋白(放射免疫分析)和核磁共振结果与侵入性检查结果进行比较,以检测肝组织中的金属沉积(原子吸收浓度、光学或电子显微镜检测)。95%的病例观察到肝脏铁过载。所有治疗均使疾病完全恢复正常,尽管放血组所需时间略长。我们可以得出结论,去铁治疗在PCT中非常有效,考虑到铁沉着症和肝损伤总是伴随该疾病,这些治疗在此类病例中被推荐为首选。

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Porphyria cutanea tarda: a unique iron-related disorder.迟发性皮肤卟啉症:一种独特的铁相关疾病。
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