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迟发性皮肤卟啉症(PCT)是一种因肝脏尿卟啉原脱酸酶缺乏,导致肝脏血红素生物合成障碍,以皮肤光敏感性增加为特点的疾病。常与肝脏铁质沉着、酒精性肝脏疾病或外源性雌激素有关。血液透析(HD)性PCT是由于卟啉水平显著升高,出现严重的迟发性皮肤损害。这类病人的治疗非常困难,因为使用氯喹虽能将卟啉从肝脏排出,但不能被透析所清除;由于绝大多数病人有终末期肾性贫血,因此,静脉切开放血术也不能作为一种常规疗法。重组人红细胞生成素(R-HU-EPO)已用来治疗终末期肾性贫血,它能动员储存铁并促进其利用以合成血红素。有报道EPO疗法与放血术联合使用是一种治疗HD性PCT的有效方法。本文作者报告一例单独使用EPO疗法治疗HD
Late-onset cutaneous porphyria (PCT) is a disorder characterized by an increase in the photosensitivity of the skin due to a deficiency of hepatic uroporphyrinogenase in the liver, a disorder of the heme biosynthesis in the liver. Often associated with liver iron deposition, alcoholic liver disease or exogenous estrogen. Hemodialysis (HD) PCT is due to a significant increase in porphyrin levels with severe delayed skin lesions. The treatment of these patients is very difficult because of the use of chloroquine porphyrin can be discharged from the liver, but can not be cleared by dialysis; Since the vast majority of patients with end-stage renal anemia, therefore, phlebotomy can not be as a Kind of conventional therapy. Recombinant human erythropoietin (R-HU-EPO) has been used to treat end-stage renal anemia, which mobilizes stored iron and promotes its utilization for the synthesis of heme. It has been reported that the combination of EPO and blood transfusion is an effective method to treat HD sex PCT. The authors report a case of EPO therapy alone for the treatment of HD