Machida Ikuo, Wakusawa Shinya, Hayashi Hisao, Suzuki Satoshi, Yano Motoyoshi
Department of Medicine, Faculty of Pharmaceutical Sciences of Hokuriku University, Ho-3, Kanagawa-machi, 920-1181 Kanazawa, Japan.
J Gastroenterol. 2004 Sep;39(9):896-9. doi: 10.1007/s00535-004-1407-9.
Direct-type hyperbilirubinemia in Dubin-Johnson syndrome is due to the genetic dysfunction of multidrug resistance protein 2. However, serum bilirubin levels may fluctuate as a result of acquired conditions. Iron-reduction therapy by venesection, an alternative to interferon, was performed in a 55-year-old male patient with Dubin-Johnson syndrome complicated by hepatitis C virus-positive chronic liver disease and hepatic iron overload. His pretreatment serum total bilirubin was 10.2 mg/dl, with a dominant direct fraction. The treatment induced a significant reduction in serum total bilirubin, although it remained as high as 7.9 mg/dl. A negative correlation between serum total bilirubin and cumulative bled volume suggested that venesection could suppress bilirubin production from aged erythrocytes. The hepatic iron overload was distributed in hepatocyte lysosomes with Dubin-Johnson granules; thus, it seems that iron removal from the lysosomal granules may also help to reduce serum bilirubin. In conclusion, deep jaundice in a patient with Dubin-Johnson syndrome complicated by hepatitis C virus-positive chronic liver disease and iron overload was partially improved by iron-reduction therapy.
杜宾-约翰逊综合征中的直接型高胆红素血症是由于多药耐药蛋白2的基因功能障碍所致。然而，血清胆红素水平可能因后天因素而波动。对一名55岁男性杜宾-约翰逊综合征患者进行了静脉放血铁减少疗法，该患者合并丙型肝炎病毒阳性慢性肝病和肝铁过载，此疗法可替代干扰素。他治疗前的血清总胆红素为10.2mg/dl，以直接胆红素为主。尽管治疗后血清总胆红素仍高达7.9mg/dl，但治疗使其显著降低。血清总胆红素与累积放血量之间呈负相关，提示静脉放血可抑制衰老红细胞产生胆红素。肝铁过载分布于含有杜宾-约翰逊颗粒的肝细胞溶酶体中；因此，从溶酶体颗粒中去除铁似乎也有助于降低血清胆红素。总之，对于合并丙型肝炎病毒阳性慢性肝病和铁过载的杜宾-约翰逊综合征患者，铁减少疗法部分改善了其深度黄疸。
