尽管在新生儿中已有病例报道,但 DJS 通常在青春期后确诊。[10]Kondo T, Yagi R. Dubin-Johnson syndrome in a neonate. N Engl J Med. 1975;292:1028-1029.http://www.ncbi.nlm.nih.gov/pubmed/1117948?tool=bestpractice.com[11]Nakata F, Oyanagi K, Fujiwara M, et al. Dubin-Johnson syndrome in a neonate. Eur J Paediatr. 1979;132:299-301.http://www.ncbi.nlm.nih.gov/pubmed/520367?tool=bestpractice.com[12]Haimi-Cohen Y, Merlob P, Marcus-Eidlits T, et al. Dubin-Johnson syndrome as a cause of neonatal jaundice: the importance of coproporphyrins investigation. Clin Pediatr (Phil). 1998;37:511-513.http://www.ncbi.nlm.nih.gov/pubmed/9729708?tool=bestpractice.com[13]Tsai WH, Teng RJ, Chu JS, et al. Neonatal Dubin-Johnson syndrome. J Paediatr Gastroenterol Nutr. 1994;18:253-254.http://www.ncbi.nlm.nih.gov/pubmed/8014778?tool=bestpractice.com确诊的年龄通常在 10 至 30 岁之间。[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总的来说,患病率极低,但比罗托综合征更常见。罗托综合征也是一种表现为高结合胆红素血症的遗传性疾病。没有准确的患病率数据。全球所有种族、国籍和民族以及男女性均有对 DJS 病例的描述,但该病以男性为主,且发病年龄较早。公认伊朗犹太人的患病率最高 (1/1300),且集中于家族中。摩洛哥犹太人的患病率几乎一样高,反映了这些人群在 2000 至 2500 年前的分化情况。[14]Shani M, Seligsohn V, Gilon E, et al. Dubin-Johnson syndrome in Israel: clinical, laboratory and genetic aspects of 101 cases. Q J Med. 1970;39:549-567.http://www.ncbi.nlm.nih.gov/pubmed/5532959?tool=bestpractice.com[15]Zlotogora J. Hereditary disorders among Iranian Jews. Am J Med Genet. 1995;58:32-37.http://www.ncbi.nlm.nih.gov/pubmed/7573153?tool=bestpractice.com[16]Mor-Cohen R, Zivelin A, Fromovich-Amit Y, et al. Age estimates of ancestral mutations causing factor VII deficiency and Dubin-Johnson syndrome in Iranian and Moroccan Jews are consistent with ancient Jewish migrations. Blood Coagul Fibrinolysis. 2007;18:139-144.http://www.ncbi.nlm.nih.gov/pubmed/17287630?tool=bestpractice.com