案例
一名 20 岁男性因咽喉痛、发热和寒战且 2 日后出现黄疸而前来就诊。他报告过去 5 年内有间歇性黄疸病史。青霉素治疗后发热、寒战和咽喉痛症状消失,但黄疸一直持续。他无其它既往病史,但一位家庭成员曾有类似的间歇性黄疸发作经历。
其他表现
患者报告腹部隐痛不适和疲乏的情况并不少见,然而这似乎并不代表任何严重的病变,可能与焦虑和对黄疸的详尽检查有关。[2]Sprinz H, Nelson RS. Persistent nonhemolytic hyperbilirubinemia associated with lipochrome-like pigment in liver cells: report of 4 cases. Ann Intern Med. 1954;41:952-962.http://www.ncbi.nlm.nih.gov/pubmed/13208040?tool=bestpractice.com[3]Dubin IN. Chronic idiopathic jaundice: a review of 50 cases. Am J Med. 1958;24:268-292.http://www.ncbi.nlm.nih.gov/pubmed/13508683?tool=bestpractice.com尽管有时可观察到肝肿大,但包括胆汁酸在内的肝功能检查 (Liver Function Test, LFT) 正常,[4]Javitt NB, Kondo T, Kuchiba K. Bile acid secretion in Dubin-Johnson syndrome. Gastroenterology. 1978;75:931-932.http://www.ncbi.nlm.nih.gov/pubmed/700338?tool=bestpractice.com并且没有溶血的证据。[1]Dubin IN, Johnson FB. Chronic idiopathic jaundice with unidentified pigment in the liver cells: a new clinicopathologic entity with report of 12 cases. Medicine. 1954;33:155-197.http://www.ncbi.nlm.nih.gov/pubmed/13193360?tool=bestpractice.com[3]Dubin IN. Chronic idiopathic jaundice: a review of 50 cases. Am J Med. 1958;24:268-292.http://www.ncbi.nlm.nih.gov/pubmed/13508683?tool=bestpractice.com血清胆红素常会出现波动,偶尔胆红素检测值可能在正常范围内。与真正胆汁淤积的相关症状相比,该病无瘙痒表现。该病可呈渐进性或急性进展,常由并发疾病、酗酒、口服避孕药或妊娠诱发。[5]Rastogi A, Krishnani N, Pandey R. Dubin-Johnson syndrome: a clinicopathologic study of twenty cases. Indian J Pathol Microbiol. 2006;49:500-504.http://www.ncbi.nlm.nih.gov/pubmed/17183837?tool=bestpractice.com已有报道称 DJS 女性患者妊娠失败的发生率高,但尚不清楚与此相关的原因。[6]Seligsohn U, Shani M. The Dubin-Johnson syndrome and pregnancy. Acta Hepatogastroenterol. 1977;24:167-169.http://www.ncbi.nlm.nih.gov/pubmed/883465?tool=bestpractice.com[7]Di Zoglio JD, Cardillo E. The Dubin-Johnson syndrome and pregnancy. Obstet Gynecol. 1973;42:560.http://www.ncbi.nlm.nih.gov/pubmed/4742659?tool=bestpractice.com慢性胆囊炎和胆石病也与该病存在一定的关联,但这也可能只是一种巧合。[5]Rastogi A, Krishnani N, Pandey R. Dubin-Johnson syndrome: a clinicopathologic study of twenty cases. Indian J Pathol Microbiol. 2006;49:500-504.http://www.ncbi.nlm.nih.gov/pubmed/17183837?tool=bestpractice.com[8]Skornick YG, Klausner JM, Lelcuk S, et al. Cholelithiasis in Dubin-Johnson syndrome. Surg Gynecol Obstet. 1983;157:447-449.http://www.ncbi.nlm.nih.gov/pubmed/6635916?tool=bestpractice.com[9]Sotelo-Avila C, Danis RK, Krafcik J, et al. Cholecystitis in a 17-year-old boy with recurrent jaundice since childhood. J Pediatr. 1988;112:668-674.http://www.ncbi.nlm.nih.gov/pubmed/3351696?tool=bestpractice.com