[Dubin-Johnson syndrome: molecular basis and pathogenesis].
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.
Mzabi-Regaya
Sabah
S
Service d'Anatomie pathologique, Hôpital Mongi Slim, Tunisie.
Chadli-Debbiche
Aschraf
A
Ben Brahim
Ehsen
E
Gritli
Sami
S
Goutallier-Ben Fadhel
Carole
C
Khalfallah
Mohamed Tahar
MT
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