Mayatepek E, Lehmann W D
Department of General Pediatrics, University Children's Hospital, University of Heidelberg, Germany.
Clin Chim Acta. 1996 May 30;249(1-2):37-46. doi: 10.1016/0009-8981(95)06256-4.
The Dubin-Johnson syndrome (DJS) is characterized by a hereditary conjugated hyperbilirubinemia and a typical dark pigment accumulation in liver parenchymal cells. In the present study the renal excretion of leukotrienes in five patients with histologically established DJS and five age- and sex-matched healthy subjects was investigated. Endogenous urinary leukotrienes were separated by high-performance liquid chromatography and subsequently quantified by immunoassays and gas chromatography-mass spectrometry. Patients with DJS excreted significantly (P < 0.01) greater amounts of cysteinyl leukotriene, LTE4 (8-fold), the omega-oxidation product omega-carboxy-LTE4 (15-fold) and the beta-oxidation metabolite omega-carboxy-tetranor-LTE3 (26-fold) into urine than healthy controls. These results imply that in DJS leukotriene elimination into bile is defective, leading to a compensatory renal leukotriene elimination and a typical excretion pattern of urinary leukotriene metabolites. Analysis of endogenous urinary leukotrienes seems to be a new approach to the noninvasive diagnosis of this disease.
杜宾-约翰逊综合征（DJS）的特征是遗传性结合胆红素血症以及肝实质细胞中典型的深色色素沉着。在本研究中，对5例经组织学确诊为DJS的患者以及5名年龄和性别匹配的健康受试者的白三烯肾排泄情况进行了调查。内源性尿白三烯通过高效液相色谱法分离，随后通过免疫测定法和气相色谱-质谱法进行定量。与健康对照组相比，DJS患者尿中半胱氨酰白三烯LTE4的排泄量显著增加（P < 0.01）（8倍），ω-氧化产物ω-羧基-LTE4（15倍）以及β-氧化代谢产物ω-羧基-四去甲-LTE3（26倍）。这些结果表明，在DJS中，白三烯向胆汁中的排泄存在缺陷，导致肾对白三烯的排泄出现代偿，以及尿白三烯代谢产物的典型排泄模式。内源性尿白三烯分析似乎是一种用于该疾病无创诊断的新方法。
