title: [Dubin-Johnson syndrome: molecular basis and pathogenesis].
abstract: The Dubin-Johnson syndrome (DJS) is an autosomal recessive liver disorder characterized by a chronic conjugated hyperbilirubinemia a dark greenish appearance of liver tissue, a double peaked sulfobromophthalein clearance curve, and a characteristic lysosomal accumulation of black pigment "melanine-like" in the hepatocytes. Laboratory datas indicated an increased urinary excretion of coproporphrin isomer I and leukotriene metabolites. In an effort to understand the morphological pattern and the pathogenesis of this disease we reviewed four cases of DJS.
authors.affiliation: Service d'Anatomie pathologique, Hôpital Mongi Slim, Tunisie.
authors.forename: Sabah
authors.initials: S
authors.lastname: Mzabi-Regaya
authors.forename: Aschraf
authors.initials: A
authors.lastname: Chadli-Debbiche
authors.forename: Ehsen
authors.initials: E
authors.lastname: Ben Brahim
authors.forename: Sami
authors.initials: S
authors.lastname: Gritli
authors.forename: Carole
authors.initials: C
authors.lastname: Goutallier-Ben Fadhel
authors.forename: Mohamed Tahar
authors.initials: MT
authors.lastname: Khalfallah
mesh_terms: D000293:Adolescent; D000328:Adult; D003937:Diagnosis, Differential; D005260:Female; D022781:Hepatocytes / Q000473:pathology; D006801:Humans; D006932:Hyperbilirubinemia / Q000209:etiology; D007566:Jaundice, Chronic Idiopathic / Q000235:genetics* / Q000503:physiopathology*; D008297:Male