Wolff C, Stella A M, Armas R, Parraguez A, Silva H, Batlle A M
Departamento de Medicina Occidente, Universidad de Chile, Hospital San Juan de Dios, Santiago, Chile.
Rev Med Chil. 1998 Mar;126(3):245-50.
Porphyria cutanea tarda (PCT) is due to a partial defect of hepatic uroporphyrinogen decarboxylase (URO-D). In the hereditary form, both hepatic and erythrocytic enzymes are altered, whereas in the acquired form, only the hepatic enzyme fails. There is a high prevalence of hepatitis C virus infection in patients with PCT, specially in those without family history of the disease.
To study erythrocytic URO-D activity in order to find out whether hepatitis C virus infection is associated to the acquired form of PCT or unveils an inactive hereditary form.
URO-D activity was measured in red blood cells of normal controls, hepatitis C virus carriers without symptoms of PCT and patients with PCT, with and without family history of the disease, with and without anti hepatitis C virus antibodies.
URO-D activity was similar in normal controls, patients with chronic liver disease associated to hepatitis C virus, and in patients with PCT without family history of the disease with and without hepatitis C virus antibodies. URO-D activity was lower in patients with PCT and family history of the disease, with and without hepatitis C virus antibodies.
PCT in patients with hepatitis C virus infection is due to an acquired alteration of hepatic URO-D. Hepatitis C virus does not modify erythrocytic URO-D.
迟发性皮肤卟啉症(PCT)是由于肝尿卟啉原脱羧酶(URO-D)部分缺陷所致。在遗传性形式中,肝和红细胞中的酶均发生改变,而在获得性形式中,仅肝酶功能异常。PCT患者中丙型肝炎病毒感染的患病率很高,尤其是在那些无该病家族史的患者中。
研究红细胞URO-D活性,以确定丙型肝炎病毒感染是否与PCT的获得性形式相关,或是否揭示一种无活性的遗传性形式。
测定正常对照、无症状的丙型肝炎病毒携带者以及有或无该病家族史、有或无抗丙型肝炎病毒抗体的PCT患者红细胞中的URO-D活性。
正常对照、与丙型肝炎病毒相关的慢性肝病患者以及无该病家族史且有或无丙型肝炎病毒抗体的PCT患者的URO-D活性相似。有该病家族史且有或无丙型肝炎病毒抗体的PCT患者的URO-D活性较低。
丙型肝炎病毒感染患者的PCT是由于肝URO-D的获得性改变所致。丙型肝炎病毒不会改变红细胞URO-D。