Erythropoietin treatment in the neuropsychiatric porphyrias.
Erythropoietin treatment in the neuropsychiatric porphyrias.
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促红细胞生成素治疗神经精神性卟啉症。
DOI：
10.1016/j.cccn.2003.07.015
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发表时间：
2003
期刊：
影响因子：
--
通讯作者：
I. Macdougall
中科院分区：
文献类型：
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作者：
A. Winkler;T. Peters;J. Marsden;A. Deacon;Georgina Chandler;I. Macdougall
背景在以前的报告中，31例神经精神性卟啉症患者进行了研究，其中9例贫血与不适当的低血清促红细胞生成素水平。我们也能够证明，在非卟啉患者（主要是糖尿病患者的自主神经病变）与促红细胞生成素治疗显着降低尿δ-aminolaevulinic acid levels.MethodsWe治疗6卟啉患者，其中5人贫血，与重组人促红细胞生成素（1000-2000 IU每周三次）。他们都在临床上，但不是生化缓解。每月检测全血细胞计数，包括网织红细胞和血小板，尿δ-氨基乙酰丙酸、胆色素原和总卟啉。基线血清铁蛋白，维生素B12，叶酸和C-反应蛋白水平均在正常范围内，血清肌酐不超过126 μmol/L.ResultsAfter 3个月的治疗，平均基线血红蛋白显着增加（P=0.01）。当治疗停止时，血红蛋白降低，在预治疗3个月后，血红蛋白水平达到。在促红细胞生成素治疗期间，尿δ-氨基乙酰丙酸、胆色素原和卟啉水平均趋于降低，但基线和促红细胞生成素治疗3个月之间的差异仅在胆色素原方面具有统计学显著性（p=0.03）。卟啉症的攻击的严重程度降低，生活质量的增加，在治疗过程中与erythropoietin.ConclusionWe的结论，在一些卟啉患者治疗促红细胞生成素减少尿δ-aminolaevulinic酸，胆色素原和卟啉水平的增加幸福和减少卟啉症的攻击的严重程度。
BackgroundIn a previous report, 31 patients with neuropsychiatric porphyria were studied and nine of these patients were anaemic in association with inappropriately low serum erythropoietin levels. We were also able to demonstrate that treatment with erythropoietin in non-porphyric patients (mainly diabetic patients with autonomic neuropathy) significantly reduced urinary δ-aminolaevulinic acid levels.MethodsWe treated six porphyric patients, five of whom were anaemic, with recombinant human erythropoietin (1000–2000 IU thrice weekly). They were all in clinical but not biochemical remission. Full blood count, including reticulocytes and platelets, urinary δ-aminolaevulinic acid, porphobilinogen and total porphyrins were measured monthly. Baseline serum ferritin, vitamin B12, folate and C-reactive protein levels were all within the normal range and serum creatinine did not exceed 126 μmol/l.ResultsAfter 3 months of treatment, the average baseline haemoglobin increased significantly (p=0.01). When treatment was stopped, the haemoglobin decreased and after 3 months pre-treatment, haemoglobin levels were reached. Urinary δ-aminolaevulinic acid, porphobilinogen and porphyrin levels all tended to decrease during treatment with erythropoietin, but the difference between baseline and 3 months of erythropoietin was statistically significant only for porphobilinogen (p=0.03). The severity of porphyria attacks was reduced and the quality of life increased during treatment with erythropoietin.ConclusionWe conclude that in some porphyric patients treatment with erythropoietin reduces urinary δ-aminolaevulinic acid, porphobilinogen and porphyrin levels with an increase in well-being and a reduction in the severity of porphyria attacks.