肝铁超载加去铁胺治疗迟发性皮肤卟啉症。

Dermatologica Pub Date : 1991-01-01 DOI:10.1159/000247732
E Rocchi, M Cassanelli, A Borghi, F Paolillo, M Pradelli, S Pellizzardi, A Vezzosi, E Gallo, M Baccarani Contri, E Ventura
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引用次数: 26

摘要

本文的目的是评估150例迟发性皮肤卟啉症(PCT)患者的有创性和无创性铁储存指标,并比较不同缺铁方案的有效性。通过强化放血(22例)或缓慢皮下和高静脉剂量地铁胺(分别为18例和5例)进行除铁,并研究了几个实验室参数;其中,低聚元素和尿卟啉(HPLC检测)在处理前后被考虑。将血清铁、转铁蛋白饱和度、铁蛋白(RIA)及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%的病例出现肝铁超载。所有的治疗方法都使疾病完全恢复正常,即使在静脉切开术组需要稍微多一点的时间。我们可以得出结论,含铁治疗在PCT中是非常有效的,考虑到铁沉着和肝损害总是伴随疾病,这些治疗被建议作为这类病例的首选。
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Liver iron overload and desferrioxamine treatment of porphyria cutanea tarda.

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.

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